Adrenocortical cancer (also called cancer of the adrenal cortex) is rare. Certain inherited disorders increase the risk of adrenocortical cancer. Explore the links on this page to learn more about adrenocortical cancer treatment, research, and clinical trials.
Paraganglioma forms in nerve tissue near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body.
Pheochromocytoma and paraganglioma may be benign (not cancer) or malignant (cancer).
Inheriting certain gene mutations (changes) increases the risk of pheochromocytoma and paraganglioma.
Signs and symptoms of pheochromocytoma and paraganglioma occur if too much adrenaline or noradrenaline is released into the blood.
Tests and procedures used to diagnose and stage pheochromocytoma and paraganglioma depend on the patient’s signs and symptoms and family history.
Certain factors affect prognosis (chance of recovery) and treatment options.
Pheochromocytoma forms in the adrenal gland.
Pheochromocytoma forms in the adrenal gland. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Pheochromocytoma is a tumor of the adrenal medulla.
EnlargeAnatomy of the adrenal gland. There are two adrenal glands, one on top of each kidney. The outer part of each gland is the adrenal cortex and the inner part is the adrenal medulla.
Paraganglioma forms in nerve tissue near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body.
Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of extra adrenaline and noradrenaline into the blood may cause symptoms.
EnlargeA paraganglioma is a rare tumor that often forms in nerve tissue near the carotid artery. It may also form along nerve pathways in the head and neck and in other parts of the body.
Pheochromocytoma and paraganglioma may be benign (not cancer) or malignant (cancer).
About half of all children with pheochromocytoma or paraganglioma have malignant pheochromocytoma or paraganglioma. This means that the tumor cells have spread to other parts of the body. Benign and malignant pheochromocytoma and paraganglioma require treatment because they can cause severe or life-threatening heart problems and affect many body functions.
Inheriting certain gene mutations (changes) increases the risk of pheochromocytoma and paraganglioma.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.
Having any of the following inheritedsyndromes or gene changes increases risk of pheochromocytoma and paraganglioma:
More than half of the children and adolescentsdiagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increases the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.
Signs and symptoms of pheochromocytoma and paraganglioma occur if too much adrenaline or noradrenaline is released into the blood.
Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma, paraganglioma, or other conditions.
Check with your child’s doctor if your child has any of the following:
These signs and symptoms may come and go, but high blood pressure is more likely to occur for long periods of time in young patients. These signs and symptoms may also occur with physical activity, injury, anesthesia, surgery to remove the tumor, eating foods such as chocolate and cheese, or while passing urine (if the tumor is in the bladder).
Tests and procedures used to diagnose and stage pheochromocytoma and paraganglioma depend on the patient’s signs and symptoms and family history.
Tests are done to diagnose and stage cancer. After cancer is diagnosed, more tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body. This process is called staging.
The following tests and procedures may be used:
Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Plasma-free metanephrines test: A blood test that measures the amount of metanephrines in the blood. Metanephrines are substances that are made when the body breaks down adrenaline or noradrenaline. Pheochromocytomas and paragangliomas can make large amounts of adrenaline and noradrenaline and cause high levels of metanephrines in both the blood and urine.
Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines (adrenaline or noradrenaline) released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (unusual higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma or paraganglioma.
Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines (adrenaline or noradrenaline) or metanephrines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma or paraganglioma.
PET scan: A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
Genetic testing: A laboratory test in which cells or tissue are analyzed to look for changes in genes or chromosomes. These changes may be a sign that a person has or is at risk of having a specific disease or condition. The following are genes that might be tested for in children with pheochromocytoma or paraganglioma: VHL, NF1, RET, SDHD, SDHB, SDHA, MAX, and TMEM127 genes.
Certain factors affect prognosis (chance of recovery) and treatment options.
Prognosis and treatment options depend on the following:
Whether the cancer has just been diagnosed or has recurred (come back).
Stages of Childhood Pheochromocytoma and Paraganglioma
Key Points
After pheochromocytoma or paraganglioma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
After pheochromocytoma or paraganglioma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
Sometimes childhood pheochromocytoma or paraganglioma recurs (comes back) after treatment. It may come back in the place it first formed or in other parts of the body.
There are three ways that cancer spreads in the body.
Tissue. The cancer spreads from where it began by growing into nearby areas.
Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatictumor is the same type of cancer as the primary tumor. For example, if pheochromocytoma spreads to the bone, the cancer cells in the bone are actually pheochromocytoma cells. The disease is metastatic pheochromocytoma, not bone cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
Treatment Option Overview
Key Points
There are different types of treatment for children with pheochromocytoma or paraganglioma.
Children with pheochromocytoma or paraganglioma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Four types of standard treatment are used:
Surgery
Chemotherapy
High-dose 131I-MIBG therapy
Targeted therapy
New types of treatment are being tested in clinical trials.
Treatment of pheochromocytoma and paraganglioma may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with pheochromocytoma or paraganglioma.
Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with pheochromocytoma or paraganglioma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
High-dose 131I-MIBG therapy
131I-MIBGtherapy is a treatment with high-doseradioactive iodine. The radioactive iodine is given through an intravenous (IV) line and enters the bloodstream which carries radiation directly to tumor cells. Radioactive iodine collects in pheochromocytoma and paraganglioma cells and kills them with the radiation that is given off.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. Catecholamine levels that are higher than normal can be a sign that the cancer has come back. Talk to your child’s doctor about which tests should be done and how often.
Patients with pheochromocytoma or paraganglioma need lifelong follow-up.
Treatment of Childhood Pheochromocytoma and Paraganglioma
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Recurrent Childhood Pheochromocytoma and Paraganglioma
A clinical trial that checks a sample of the patient’s tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
To Learn More About Childhood Pheochromocytoma and Paraganglioma
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood pheochromocytoma and paraganglioma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
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Anatomy of the digestive tract. The digestive tract is made up of organs that food and liquids travel through when they are swallowed, digested, absorbed, and leave the body as feces. These organs include the mouth, pharynx (throat), esophagus, stomach, small intestine, large intestine, rectum, and anus.
Childhood stomach cancer is a very rare cancer that starts in the cells lining the stomach. The stomach is an organ on the left side of the upper abdomen that digests food. The stomach is part of the digestive tract, a series of hollow, muscular organs joined in a long, twisting tube from the mouth to the anus. The digestive tract processes nutrients in foods that are eaten and helps pass waste material out of the body:
Food moves from the throat to the stomach through a tube called the esophagus.
After food enters the stomach, it is broken down by stomach muscles that mix the food and liquid with digestive juices.
After leaving the stomach, partly digested food passes into the small intestine and then into the large intestine.
The end of the large intestine, called the rectum, stores the waste from the digested food until it is pushed out of the anus during a bowel movement.
Causes and risk factors for childhood stomach cancer
Childhood stomach cancer is caused by certain changes to the way stomach cells function, especially how they grow and divide into new cells. Often, the exact cause of the cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with a risk factor will develop stomach cancer, and it will develop in some children who don’t have a known risk factor.
Risk factors for childhood stomach cancer include:
H. pylori infection. Chronic infection of the mucosal layer of the stomach with H. pylori is a risk factor for stomach cancer. This bacterium spreads from person to person through direct contact with saliva, vomit, or stool. Although many people with chronic H. pylori infections do not have symptoms, some develop stomach ulcers or inflammation of the stomach called atrophic gastritis. In some people, atrophic gastritis leads to increasingly severe changes in the stomach lining and eventually to stomach cancer or gastric MALT lymphoma. Treatment of H. pylori infections reduces the risk of these types of stomach cancer.
Talk with your child’s doctor if you think your child might be at risk of stomach cancer.
Genetic counseling for children with stomach cancer
It may not be clear from the family medical history whether a child with stomach cancer has an inherited condition that increased their risk. Genetic testing may help explain why a child develops a rare cancer or a cancer that is usually seen in adults, such as stomach cancer. Genetic counselors and other specially trained health professionals can discuss your child’s diagnosis and your family’s medical history to help you understand:
the risk of stomach cancer and other cancers for your child’s siblings
the risks and benefits of learning genetic information
Genetic counselors can also help you cope with your child’s genetic testing results, including how to discuss the results with family members.
Symptoms of childhood stomach cancer
Many children do not have symptoms of stomach cancer until the cancer has spread. It’s important to check with your child’s doctor if your child has:
stomach pain
loss of appetite
weight loss for no known reason
nausea
vomiting
constipation or diarrhea
weakness
anemia (symptoms of which may include tiredness, dizziness, fast or irregular heartbeat, shortness of breath, and pale skin)
These symptoms may be caused by conditions other than childhood stomach cancer. The only way to know is to see your child’s doctor. The doctor will ask you when the symptoms started and how often your child has been having them as a first step in making a diagnosis.
Diagnosis of childhood stomach cancer
If your child has symptoms that suggest stomach cancer, the doctor will need to find out if they are due to cancer or another condition. The doctor will ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend tests to find out if your child has stomach cancer.
The following tests and procedures are used to diagnose stomach cancer. The results will also help you and your child’s doctor plan treatment.
Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
Upper endoscopy is a procedure to look inside the esophagus, stomach, and duodenum (first part of the small intestine) to check for abnormal areas. An endoscope is passed through the mouth and down the throat into the esophagus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples (biopsy), which are checked under a microscope for signs of cancer.
Talk with your child’s doctor about what to expect during and after your child’s biopsy.
Learn about the type of information that can be found in a pathologist’s report about the cells or tissue removed during a biopsy at Pathology Reports.
Barium swallow
Barium swallow is a series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
Getting a second opinion
You may want to get a second opinion to confirm your child’s stomach cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.
Prognostic factors for childhood stomach cancer
If your child has been diagnosed with stomach cancer, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis. The prognosis can be affected by whether the cancer has spread to other parts of the body at the time of diagnosis and how well the cancer responds to treatment. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.
Stages of childhood stomach cancer
Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. There is no staging system used for childhood stomach cancer, but the tests and procedures done to diagnose the cancer are also used to help plan treatment.
Types of treatment for childhood stomach cancer
There are different types of treatment for children and adolescents with stomach cancer. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for childhood stomach cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.
The types of treatment your child might have include:
Surgery
Surgery to remove the tumor is the main treatment for stomach cancer.
Radiation therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Stomach cancer is sometimes treated with external beam radiation therapy. This type of radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Radiation therapy may be given alone or with other treatments, such as chemotherapy.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Chemotherapy may be given alone or with other types of treatment, such as radiation therapy.
For stomach cancer, the chemotherapy is injected into a vein. When given this way, the drugs enter the bloodstream to reach cancer cells throughout the body. Chemotherapy drugs used alone or in combination to treat stomach cancer in children are:
Other chemotherapy drugs not listed here may also be used.
Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Treatment of newly diagnosed stomach cancer in children may include:
surgery to remove the cancer and some healthy tissue around it
surgery to remove as much of the cancer as possible, followed by radiation therapy and chemotherapy
If your child’s cancer comes back after treatment, their doctor will talk with you about what to expect and next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Side effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
To learn more about side effects that begin during treatment for cancer, visit Side Effects.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer) or other conditions
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments.
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Coping with cancer
When your child has cancer, every member of the family needs support. Honest and calm conversations build trust as you talk with your child and their siblings. Taking care of yourself during this difficult time is also important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.
Having a certain mutation (change) in the TP53 gene increases the risk of adrenocortical carcinoma.
Signs and symptoms of adrenocortical carcinoma include a lump or pain in the abdomen.
Tests that examine the adrenal glands are used to diagnose and stage adrenocortical carcinoma.
Certain factors affect prognosis (chance of recovery).
Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.
There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Adrenocortical carcinoma is also called adrenocortical cancer or cancer of the adrenal cortex.
EnlargeAnatomy of the adrenal gland. There are two adrenal glands, one on top of each kidney. The outer part of each gland is the adrenal cortex and the inner part is the adrenal medulla.
Most childhood adrenocorticaltumors occur during the first 5 years of life, but they may also occur during adolescence.
Having a certain mutation (change) in the TP53 gene increases the risk of adrenocortical carcinoma.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.
The risk of adrenocortical carcinoma is increased by having a mutation (change) in the TP53gene or any of the following syndromes:
Also, cancer of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make extra hormones). Most tumors of the adrenal cortex in children are functioning tumors. The extra hormones made by functioning tumors may cause certain signs or symptoms of disease and these depend on the type of hormone made by the tumor. For example, extra androgen hormone may also cause male children to develop an enlarged penis and female children to develop enlarged genitalia. Extra estrogen hormone may cause the growth of breasttissue in male children. Extra cortisol hormone may cause (hypercortisolism).
See the PDQ summary onAdrenocortical Carcinoma Treatment (Adult) for more information on the signs and symptoms of adrenocortical carcinoma.
Tests that examine the adrenal glands are used to diagnose and stage adrenocortical carcinoma.
Tests are done to diagnose and stage cancer. After cancer is diagnosed, more tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body. This process is called staging. It is important to know whether cancer has spread in order to plan the best treatment.
The following tests and procedures may be used:
Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
X-ray: An x-ray of the chest, abdomen, or bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
CT scan: A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest and abdomen. This procedure is also called nuclear magnetic resonance imaging (NMRI). EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
PET scan: A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. EnlargeAbdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
Biopsy: The removal of cells or tissues during surgery so they can be viewed under a microscope by a pathologist to check for signs of cancer.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may be checked for testosterone or estrogen. A higher than normal amount of these hormones may be a sign of adrenocortical carcinoma.
Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these substances in the urine may be a sign of disease in the adrenal cortex.
Low-dosedexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
Certain factors affect prognosis (chance of recovery).
The prognosis is good for patients who have small tumors that have been completely removed by surgery. For other patients, the prognosis depends on the following:
The size of the tumor.
How quickly the cancer is growing.
Whether there are changes in certain genes.
Whether the tumor has spread to other parts of the body, including the lymph nodes, liver, lung, kidney, or bone.
The child’s age.
Whether the covering around the tumor broke open during surgery to remove the tumor.
Whether the tumor was completely removed during surgery.
Whether the child has developed masculine traits.
Stages of Adrenocortical Carcinoma
Key Points
After adrenocortical carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
After adrenocortical carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
The process used to find out if cancer has spread to tissues near the adrenal glands or to other parts of the body is called staging. The information gathered from the staging process is used to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease.
Tissue. The cancer spreads from where it began by growing into nearby areas.
Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatictumor is the same type of cancer as the primary tumor. For example, if adrenocortical carcinoma spreads to the liver, the cancer cells in the liver are actually adrenocortical carcinoma cells. The disease is metastatic adrenocortical carcinoma, not liver cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
Treatment Option Overview
Key Points
There are different types of treatment for children with adrenocortical carcinoma.
Children with adrenocortical carcinoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Two types of standard treatment are used:
Surgery
Chemotherapy
New types of treatment are being tested in clinical trials.
Immunotherapy
Treatment for childhood adrenocortical carcinoma may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with adrenocortical carcinoma.
Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with adrenocortical carcinoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Immunotherapy
Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy.
Immune checkpoint inhibitor therapy is a type of immunotherapy that blocks certain proteins. PD-1 is a protein on the surface of T cells that helps keep the body’s immune responses in check. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors keep PD-1 and PD-L1 proteins from attaching to each other. This allows the T cells to kill cancer cells. Pembrolizumab is a PD-1 inhibitor that is being studied in the treatment of childhood adrenocortical carcinoma that is advanced or has come back after treatment.
EnlargeImmune checkpoint inhibitor. Checkpoint proteins, such as PD-L1 on tumor cells and PD-1 on T cells, help keep immune responses in check. The binding of PD-L1 to PD-1 keeps T cells from killing tumor cells in the body (left panel). Blocking the binding of PD-L1 to PD-1 with an immune checkpoint inhibitor (anti-PD-L1 or anti-PD-1) allows the T cells to kill tumor cells (right panel).
Immunotherapy uses the body’s immune system to fight cancer. This animation explains one type of immunotherapy that uses immune checkpoint inhibitors to treat cancer.
Treatment for childhood adrenocortical carcinoma may cause side effects.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer. for more information.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Recurrent Childhood Adrenocortical Carcinoma
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
To Learn More About Childhood Adrenocortical Carcinoma
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood adrenocortical carcinoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Adrenocortical Carcinoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/adrenocortical/patient/child-adrenocortical-treatment-pdq. Accessed <MM/DD/YYYY>.
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
Heart tumors (also called cardiac tumors) are rare growths that can form in any part of the heart. They can be benign (not cancerous) or malignant (cancerous). Most heart tumors in children are benign. Although benign tumors do not spread to other parts of the body, they may require treatment to stop them from causing problems with the heart’s rhythm and blood flow. Cancerous heart tumors are very rare in children. They can spread to other parts of the body and require more intensive treatment than benign tumors.
Types of benign heart tumors that may occur in a fetus, newborn, or child include:
Rhabdomyoma is a tumor that forms in muscle made up of long fibers.
Teratoma is a type of germ cell tumor. In the heart, these tumors form most often in the pericardium (the sac that covers the heart). Some teratomas are cancerous.
Fibroma is a tumor that forms in fiber-like tissue that holds bones, muscles, and other organs in place.
Histiocytoid cardiomyopathy tumor is a tumor that forms in the heart cells that control heart rhythm.
Hemangioma is a tumor that forms in the cells that line blood vessels.
Neurofibroma is a tumor that forms in the cells and tissues that cover nerves.
The most common benign heart tumors that may occur in a fetus or newborn include rhabdomyoma, myxoma, teratoma, and fibroma. Most rhabdomyomas go away on their own.
EnlargeHeart tumors are rare tumors that form in the tissues of the heart, including muscle tissue, connective tissue, and tissues that line the blood vessels, control heart rhythm, and cover the nerves of the heart. They may also form in the pericardium (the sac around the heart). Most heart tumors are benign (not cancer), but some may be malignant (cancer).
Types of cancerous heart tumors that may occur in a fetus, newborn, or child include:
Malignant teratoma is a type of germ cell tumor. In the heart, these tumors form most often in the pericardium (the sac that covers the heart). Most teratomas are not cancer.
Primary cardiac lymphoma begins in the heart and/or pericardium (the sac that covers the heart).
Rhabdomyosarcoma forms in muscle made up of long fibers.
Angiosarcoma forms in cells that line blood vessels or lymph vessels.
Undifferentiated pleomorphic sarcoma usually forms in the soft tissue, but it may also form in bone.
Leiomyosarcoma forms in smooth muscle cells.
Chondrosarcoma usually forms in bone cartilage, but very rarely can begin in the heart.
Synovial sarcoma usually forms around joints, but may very rarely form in the heart or sac around the heart.
Infantile fibrosarcoma forms in fiber-like tissue that holds bones, muscles, and other organs in place.
Causes and risk factors for childhood heart tumors
Heart tumors in children are caused by certain changes to the way heart cells function, especially how they grow and divide into new cells. Often, the exact cause of the cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. A fetus or newborn can have a higher risk of benign heart tumors if they have a genetic condition called tuberous sclerosis. Another condition, called Carney complex, might also increase a child’s risk of a type of benign heart tumor called myxoma. Not all children with these risk factors will get a benign heart tumor, and benign heart tumors may develop in some children who don’t have a known risk factor. Talk with your child’s doctor if you think your child may be at risk.
There are no known risk factors for cancerous heart tumors in children.
Symptoms of childhood heart tumors
Most children with a heart tumor will have some signs and symptoms that alert parents of a problem. It’s important to check with your child’s doctor if your child has:
a change in the heart’s normal rhythm
trouble breathing, especially when lying down
pain or tightness in the middle of the chest that feels better when sitting up
numbness or weakness of the face, arm, or leg (especially on one side of the body)
confusion or trouble speaking or understanding
trouble seeing with one or both eyes
trouble walking or feeling dizzy
loss of balance or coordination
severe headache for no known reason
These symptoms may be caused by problems other than a heart tumor. The only way to know is to see your child’s doctor.
Tests to diagnose childhood heart tumors
If your child has symptoms that suggest a heart tumor, the doctor will need to find out the cause. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with a heart tumor, the results of these tests will help you and your child’s doctor plan treatment.
The tests and procedures used to diagnose heart tumors may include:
Echocardiogram
An echocardiogram uses high-energy sound waves (ultrasound) that bounce off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).
EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
Chest x-ray
An x-ray is a type of radiation that can go through the body and make pictures. A chest x-ray is one that makes pictures of the organs and bones inside the chest.
Electrocardiogram (EKG)
An EKG is a recording of the heart’s electrical activity to check its rate and rhythm. Small pads (electrodes) are placed on the patient’s chest, arms, and legs, and are connected by wires to the EKG machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.
Cardiac catheterization
Cardiac catheterization is a procedure to look inside the blood vessels and heart for abnormal areas or cancer. A long, thin, catheter is inserted into an artery or vein in the groin, neck, or arm and threaded through the blood vessels to the heart. A sample of tissue may be removed using a special tool. A pathologist views the tissue under a microscope to look for cancer cells.
Getting a second opinion
You may want to get a second opinion to confirm your child’s diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s tumor.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor About Cancer.
Types of treatment for childhood heart tumors
Who treats children with heart tumors?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of heart tumors. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with heart tumors. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the tumor is newly diagnosed or has come back.
Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s care team before treatment begins about what to expect. For help every step of the way, see our booklet, Children with Cancer: A Guide for Parents.
Watchful waiting
Watchful waiting is closely monitoring a child’s condition without giving any treatment until signs or symptoms appear or change. This treatment may be used for rhabdomyoma (a benign tumor).
Surgery
When possible, the tumor is removed by surgery. Types of surgery that may be done include:
Surgery to remove the tumor and some healthy tissue around it.
Heart transplant. If the child is waiting for a donated heart, they may receive other treatment as needed.
Sometimes chemotherapy may be given before surgery to make a cancerous tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy.
Chemotherapy
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. For children with sarcoma, chemotherapy may be given alone or with other types of treatment to reduce the size of the tumor before surgery.
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Cancerous heart tumors are sometimes treated with external beam radiation therapy. External beam radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Radiation therapy may be used when the cancer cannot be removed by surgery.
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth of tumor cells.
Targeted therapies used to treat benign heart tumors include:
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
chemotherapy followed by surgery (which may include removing some or all of the tumor or a heart transplant) for children with sarcomas
surgery to completely remove the tumor for children with other types of heart tumors
radiation therapy for children with cancerous tumors that cannot be removed by surgery
If a cancerous tumor comes back after treatment, your child’s doctor will talk with you about what to expect and possible next steps. There may be treatment options that could shrink the tumor or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Side effects and late effects of treatment
Treatments for heart tumors can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer) or other conditions
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Coping with your child's cancer
When your child has a heart tumor, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, see:
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood cardiac (heart) tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Heart Tumors. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/cardiac/patient-child-cardiac-treatment-pdq. Accessed <MM/DD/YYYY>.
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
Thymomas and thymic carcinomas are rare tumors that form in cells on the thymus. Thymomas grow slowly and rarely spread beyond the thymus. Thymic carcinoma grows faster, often spreads to other parts of the body, and is harder to treat. Explore the links on this page to learn more about thymoma and thymic carcinoma treatment and clinical trials.
EnlargeAnatomy of the thymus gland. The thymus gland is a small organ that lies in the upper chest under the breastbone. It makes white blood cells, called lymphocytes, which protect the body against infections.
Even though thymoma and thymic carcinoma form in the same type of cell, they act differently:
Thymoma. The cancer cells look a lot like the normal cells of the thymus, grow slowly, and rarely spread beyond the thymus. A thymoma may become a thymic carcinoma over time.
Thymic carcinoma. The cancer cells do not look like the normal cells of the thymus, grow more quickly, and are more likely to spread to other parts of the body.
Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus, but they are not considered to be thymoma or thymic carcinoma.
Signs and symptoms of thymoma and thymic carcinoma include coughing and trouble breathing.
These and other signs and symptoms may be caused by thymoma and thymic carcinoma or by other conditions.
Check with your child’s doctor if your child has any of the following:
Tests that examine the thymus and chest are used to help diagnose thymoma and thymic carcinoma.
The following tests and procedures may be used:
Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
Certain factors affect prognosis (chance of recovery).
Whether the cancer is thymoma or thymic carcinoma.
Whether the cancer has spread to nearby areas or other parts of the body.
Whether the cancer was completely removed by surgery.
Whether the cancer is newly diagnosed or has recurred (come back) after treatment.
The prognosis is better when the cancer has not spread to other parts of the body. Childhood thymoma is usually diagnosed before the tumor has spread.
Stages of Thymoma and Thymic Carcinoma
Key Points
After thymoma or thymic carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
After thymoma or thymic carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
The process used to find out if thymoma or thymic carcinoma has spread from the thymus to nearby areas or other parts of the body is called staging. Thymoma and thymic carcinoma may spread to the lungs, liver, kidneys, or the lining around the lungs and heart. The results of tests and procedures done to diagnose thymoma or thymic carcinoma are used to help make decisions about treatment.
Sometimes childhood thymoma or thymic carcinoma recurs (comes back) after treatment.
There are three ways that cancer spreads in the body.
Tissue. The cancer spreads from where it began by growing into nearby areas.
Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatictumor is the same type of cancer as the primary tumor. For example, if thymic carcinoma spreads to the lung, the cancer cells in the lung are actually thymic carcinoma cells. The disease is metastatic thymic carcinoma, not lung cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
Treatment Option Overview
Key Points
There are different types of treatment for children with thymoma or thymic carcinoma.
Children with thymoma or thymic carcinoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Five types of standard treatment are used:
Surgery
Radiation therapy
Chemotherapy
Hormone therapy
Targeted therapy
New types of treatment are being tested in clinical trials.
Treatment of thymoma and thymic carcinoma may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with thymoma or thymic carcinoma.
Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with thymoma or thymic carcinoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
Surgery to remove the cancer is the main treatment for thymoma and thymic carcinoma. However, thymic carcinoma can rarely be completely removed by surgery and is likely to recur (come back) after treatment.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
Hormone therapy
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances that are made by glands in the body and flow through the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs may be used to reduce the production of hormones or block them from working. Hormone therapy using corticosteroids or octreotide may be used to treat thymoma.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Tyrosine kinase inhibitors: These targeted therapy drugs block signals needed for tumors to grow. Sunitinib is used to treat thymoma and thymic carcinoma that did not respond to other treatments.
Targeted therapy is being studied for the treatment of thymoma and thymic carcinoma that has recurred (come back).
New types of treatment are being tested in clinical trials.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Recurrent Thymoma and Thymic Carcinoma
A clinical trial that checks a sample of the patient’s tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood thymoma and thymic carcinoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Thymoma and Thymic Carcinoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/thymoma/patient/child-thymoma-treatment-pdq. Accessed <MM/DD/YYYY>.
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
Childhood Esophageal Cancer (PDQ®)–Patient Version
What is childhood esophageal cancer?
Childhood esophageal cancer is a rare type of cancer that forms in the esophagus.
The esophagus is the hollow, muscular tube that moves food and drink from the throat to the stomach. The wall of the esophagus is made up of several layers of tissue, including mucous membrane, muscle, and connective tissue.
EnlargeAnatomy of the esophagus. The esophagus is a hollow, muscular tube that moves food and liquid from the pharynx (throat) to the stomach. The wall of the esophagus is made up of several layers of tissue, including the mucosa layer, thin muscle layer, submucosa layer, thick muscle layer, and connective tissue layer.
There are two common forms of esophageal cancer.
Squamous cell carcinoma of the esophagus is the most common esophageal tumor in children. This cancer begins in the thin, flat cells called squamous cells that line the inside of the esophagus.
Adenocarcinoma of the esophagus is a less common type of esophageal tumor in children. It begins in the glands that secrete mucus in the esophagus.
Causes and risk factors for childhood esophageal cancer
Esophageal cancer in children is caused by certain changes in the way cells in the esophagus function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop esophageal cancer. And it will develop in some children who don’t have a known risk factor.
Known risk factors for childhood esophageal cancer include:
swallowing chemicals, which may burn the esophagus and cause cell changes that may lead to cancer
Talk with your child’s doctor if you think your child may be at risk.
Symptoms of childhood esophageal cancer
Children with esophageal cancer may not have symptoms until the tumor has grown large or spread. It’s important to check with your child’s doctor if your child:
has trouble swallowing
loses weight
feels tired and weak
has indigestion and heartburn
has reflux
has nausea or vomiting
These symptoms may be caused by problems other than cancer. The only way to know is to see your child’s doctor.
Tests to diagnose childhood esophageal cancer
If your child has symptoms that suggest esophageal cancer, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with esophageal cancer, the results of these tests will help you and your child’s doctor plan treatment.
The tests used to diagnose esophageal cancer may include:
Chest x-ray
An x-ray is a type of radiation that can go through the body and make pictures. A chest x-ray is one that makes pictures of the organs and bones inside the chest.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest and abdomen. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
PET scan (positron emission tomography scan)
A PET scan uses a small amount of radioactive sugar (radioactive glucose) that is injected into a vein. The PET scanner rotates around the body to make detailed, computerized pictures of areas inside the body where the glucose is taken up. Because cancer cells often take up more glucose than normal cells, the pictures can be used to find cancer cells in the body.
EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of the esophagus. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Ultrasound exam
A test that uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Barium swallow
Barium swallow is a series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
Esophagoscopy
An esophagoscopy is a procedure to look inside the esophagus to check for abnormal areas. An esophagoscope is inserted through the mouth or nose and down the throat into the esophagus. An esophagoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A biopsy is usually done during an esophagoscopy. Sometimes a biopsy shows changes in the esophagus that are not cancer but may lead to cancer.
Bronchoscopy
A bronchoscopy is a procedure to look inside the trachea and large airways in the lungs for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Thoracoscopy
A thoracoscopy is a surgical procedure to look at the organs inside the chest to check for abnormal areas. A cut (incision) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. Sometimes this procedure is used to remove part of the esophagus or lung.
Immunohistochemistry
Immunohistochemistry is a laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to the antigen in the sample of the patient’s cells, the enzyme or dye is activated, and the antigen can then be seen under a microscope.
Getting a second opinion
You may want to get a second opinion to confirm your child’s cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.
Who treats children with esophageal cancer?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for esophageal cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with esophageal cancer. To decide on a treatment plan for your child, you and their care team will think about many factors, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s care team about what to expect before treatment begins. For help every step of the way, see our booklet, Children with Cancer: A Guide for Parents.
Treatment of esophageal cancer in children might include:
Radiation therapy, which uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Internal radiation therapy is used to treat esophageal cancer. For this treatment, a plastic or metal tube is passed through your child’s mouth into the esophagus. A machine that is outside the body has a special tool that is placed in the tube to send radiation toward the cancer. To learn more, visit Radiation Therapy to Treat Cancer.
Chemotherapy (also called chemo), which uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given in different ways. To learn more about how chemotherapy works, how it is given, common side effects, and more, visit Chemotherapy to Treat Cancer.
Surgery to remove as much of the tumor as possible.
If your child’s cancer comes back after treatment, their doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Prognostic factors for childhood esophageal cancer
If your child has been diagnosed with esophageal cancer, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis.
Your child’s prognosis depends on:
the type of esophageal cancer (squamous cell or adenocarcinoma)
whether the cancer was completely removed by surgery
whether the cancer has spread to other areas of the body
whether the cancer is newly diagnosed or has recurred (come back)
The prognosis is better if the cancer can be completely removed during surgery and if it has not spread to other areas of the body.
No two people are alike, and responses to treatment can vary greatly. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.
Side effects and late effects of cancer treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems, such as narrowing of the esophagus
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer) or other problems
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition changes or if the cancer comes back.
Coping with your child's cancer
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer.
Related resources
For more childhood cancer information and other general cancer resources, visit:
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood esophageal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Esophageal Cancer. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/esophageal/patient/child-esophageal-treatment-pdq. Accessed <MM/DD/YYYY>.
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
Pleuropulmonary blastoma is a rare and aggressive type of childhood cancer that forms in the lungs, the tissue that covers the lungs, and the inside wall of the chest cavity called the pleura. It can also form in the heart, aorta, pulmonary artery (the blood vessel that takes blood from the heart to the lungs), and the diaphragm (the main breathing muscle below the lungs). Pleuropulmonary blastoma mainly occurs in younger children, typically before the age of 5 years.
EnlargePleuropulmonary blastoma is a rare, fast-growing cancer that forms in the tissues of the lung and pleura (the thin layer of tissue that covers the lungs and lines the inside of the chest). It may also form in the organs between the lungs, including the heart, aorta, and pulmonary artery, or in the diaphragm (the main breathing muscle below the lungs). Pleuropulmonary blastoma usually occurs in infants and young children.
There are four types of pleuropulmonary blastoma:
Type I tumors are cyst-like lung tumors. They are most common in children younger than 3 years and children have a good chance of recovery. After treatment, sometimes a Type I tumor may recur as a Type II or III tumor.
Type Ir tumors are Type I tumors that have gotten smaller (regressed) or have not grown or spread.
Type II tumors are cyst-like with some solid parts. They are found more often in children older than 2 years. Type II tumors sometimes spread to the brain or other parts of the body.
Type III tumors are solid tumors. They are found more often in children older than 2 years. Type III tumors often spread to the brain or other parts of the body.
Causes and risk factors for pleuropulmonary blastoma
Pleuropulmonary blastoma is caused by certain changes to the way lung cells function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Children with a change in the DICER1 gene or a family history of DICER1 syndrome may be at an increased risk of pleuropulmonary blastoma. DICER1 syndrome is a rare genetic disorder that may cause a goiter, polyps in the colon, and tumors of the ovary, cervix, testicle, kidney, brain, eye, and lining of the lung. Not every child with these risk factors will develop a pleuropulmonary blastoma. And it will develop in some children who don’t have a known risk factor.
Talk with your child’s doctor if you think your child may be at risk.
Genetic counseling for children with pleuropulmonary blastoma
It may not be clear from the family medical history whether your child’s pleuropulmonary blastoma is part of an inherited condition related to a change in the DICER1 gene. Genetic counseling can assess the likelihood that your child’s cancer is inherited and whether genetic testing is needed. Genetic counselors and other specially trained health professionals can discuss your child’s diagnosis and your family’s medical history to help you understand:
the options for testing for the DICER1 gene
the risk of other cancers for your child
the risk of pleuropulmonary blastoma or other cancers for your child’s siblings
the risks and benefits of learning genetic information
Genetic counselors can also help you cope with your child’s genetic testing results, including how to discuss the results with family members. They can also advise you about whether other members of your family should receive genetic testing.
These symptoms may be caused by problems other than a pleuropulmonary blastoma. The only way to know is to see your child’s doctor.
Tests to diagnose pleuropulmonary blastoma
If your child has symptoms that suggest a pleuropulmonary blastoma, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with pleuropulmonary blastoma, the results of these tests will help you and your child’s doctor plan treatment.
The tests used to diagnose pleuropulmonary blastoma may include:
Chest x-ray
Chest x-ray is a type of radiation that can go through the body and make pictures of the organs and bones inside the chest.
CT scan
CT scan (CAT scan) uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest and abdomen. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
PET scan
PET scan (positron emission tomography scan) uses a small amount of radioactive sugar (also called radioactive glucose) that is injected into a vein. The PET scanner rotates around the body and makes pictures of where sugar is being used by the body. Cancer cells show up brighter in the pictures because they are more active and take up more sugar than normal cells do.
EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas in the body, such as the head. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Echocardiogram
Echocardiogram uses high-energy sound waves (ultrasound) that bounce off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
Ultrasound
Ultrasound uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Bronchoscopy
Bronchoscopy is a procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Thoracoscopy
Thoracoscopy is a type of surgery used to look inside the chest for abnormal areas. For this surgery, a thoracoscope is inserted into the chest through a cut made between two ribs. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. Sometimes part of the esophagus or lung is also removed. A thoracotomy may be done if the thoracoscope cannot reach certain tissues, organs, or lymph nodes. In this procedure, a larger incision is made between the ribs and the chest is opened.
DICER1 genetic testing
DICER1 genetic testing examines a sample of blood or saliva for changes in the DICER1 gene.
Immunohistochemistry
Immunohistochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and help tell one type of cancer from another type.
Getting a second opinion
You may want to get a second opinion to confirm your child’s cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the genetic test report, pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, visit Questions to Ask Your Doctor About Cancer.
Types of treatment for pleuropulmonary blastoma
Who treats children with pleuropulmonary blastoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of pleuropulmonary blastoma. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and also specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children with pleuropulmonary blastoma. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as where the cancer is located and your child’s age and overall health.
Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
For all types of pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer. Chemotherapy may be given to shrink the tumor before surgery for patients with Types II or III pleuropulmonary blastoma.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment. Chemotherapy for pleuropulmonary blastoma is injected into a vein. When given this way, the drugs enter the bloodstream and can reach cancer cells throughout the body.
Chemotherapy drugs used alone or in combination to treat pleuropulmonary blastoma include:
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Treatment of pleuropulmonary blastoma may include:
For Type I and Type Ir pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer.
For Type II and Type III pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer. Chemotherapy may be given before surgery to shrink the tumor.
If the cancer comes back after treatment, your child’s doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Prognostic factors for pleuropulmonary blastoma
If your child has been diagnosed with pleuropulmonary blastoma, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis. The prognosis can be affected by the type of pleuropulmonary blastoma, whether the cancer has spread to other parts of the body at the time of diagnosis, and whether the cancer was completely removed by surgery.
No two people are alike, and responses to treatment can vary greatly. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.
Side effects and late effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer) or other conditions
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, visit Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, visit:
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