Laryngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the larynx.

The larynx is a part of the throat, between the base of the tongue and the trachea. The larynx contains the vocal cords, which vibrate and make sound when air is directed against them. The sound echoes through the pharynx, mouth, and nose to make a person’s voice.

There are three main parts of the larynx:

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Most laryngeal cancers form in squamous cells, the thin, flat cells lining the inside of the larynx.

Laryngeal cancer is a type of head and neck cancer.

Use of tobacco products and drinking too much alcohol can affect the risk of laryngeal cancer.

Anything that increases a person’s chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop laryngeal cancer, and it will develop in people who don’t have any known risk factors. Talk with your doctor if you think you may be at risk.

Signs and symptoms of laryngeal cancer include a sore throat and ear pain.

These and other signs and symptoms may be caused by laryngeal cancer or by other conditions. Check with your doctor if you have any of the following:

Tests that examine the throat and neck are used to help diagnose and stage laryngeal cancer.

In addition to asking about your personal and family health history, your doctor may perform the following tests and procedures:

Certain factors affect prognosis (chance of recovery) and treatment options.

Prognosis depends on the following:

Treatment options depend on the following:

Smoking tobacco and drinking alcohol decrease the effectiveness of treatment for laryngeal cancer. Patients with laryngeal cancer who continue to smoke and drink are less likely to be cured and more likely to develop a second tumor. After treatment for laryngeal cancer, frequent and careful follow-up is important.

After laryngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the larynx or to other parts of the body.

The process used to find out if cancer has spread within the larynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of some of the tests used to diagnose laryngeal cancer are often also used to stage the disease.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

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.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if laryngeal cancer spreads to the lung, the cancer cells in the lung are actually laryngeal cancer cells. The disease is metastatic laryngeal cancer, not lung cancer.

The following stages are used for laryngeal cancer:

Stage III

In stage III, cancer has formed in the supraglottis, glottis, or subglottis area of the larynx:

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In stage III cancer of the supraglottis:

• cancer is in the larynx only and the vocal cords do not work, and/or cancer has spread near or through the inner part of the thyroid cartilage. Cancer may have also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer is in one area of the supraglottis and the vocal cords work normally. Cancer has spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer is in more than one area of the supraglottis or has spread to the area at the base of the tongue or to tissues near the vocal cords. The vocal cords work normally. Cancer has also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller.

In stage III cancer of the glottis:

• cancer is in the larynx only and the vocal cords do not work, and/or cancer has spread near or through the inner part of the thyroid cartilage. Cancer may have also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer is in one or both vocal cords and the vocal cords work normally. Cancer has spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer has spread to the supraglottis, subglottis, or both, and/or the vocal cords do not work normally. Cancer has also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller.

In stage III cancer of the subglottis:

• cancer is in the larynx only and the vocal cords do not work, and/or cancer has spread near or through the inner part of the thyroid cartilage. Cancer may have also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer is in the subglottis only. Cancer has spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller; or
• cancer has spread to one or both vocal cords and the vocal cords may not work normally. Cancer has also spread to one lymph node on the same side of the neck as the primary tumor and the lymph node is 3 centimeters or smaller.

After surgery, the stage of the cancer may change and more treatment may be needed.

If the cancer is removed by surgery, a pathologist will examine a sample of the cancer tissue under a microscope. Sometimes, the pathologist’s review will result in a change to the stage of the cancer and more treatment after surgery.

Laryngeal cancer can recur (come back) after it has been treated.

The cancer may come back in the larynx or in other parts of the body, such as lungs, liver, or bone. It is most likely to come back in the first 2 to 3 years.

There are different types of treatment for patients with laryngeal cancer.

Different types of treatment are available for patients with laryngeal cancer. 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. 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.

The following types of treatment are used:

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.

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Radiation therapy may work better in patients who have stopped smoking before beginning treatment. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the body may be done before and after therapy to make sure the thyroid gland is working properly.

Hyperfractionated radiation therapy may be used to treat laryngeal cancer. Hyperfractionated radiation therapy is radiation treatment in which a smaller than usual total daily dose of radiation is divided into two doses and the treatments are given twice a day. Hyperfractionated radiation therapy is given over the same period of time (days or weeks) as standard radiation therapy. New types of radiation therapy are being studied in the treatment of laryngeal cancer.

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.

• PD-1 and PD-L1 inhibitor therapy: 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. Nivolumab and pembrolizumab are types of PD-1 inhibitors used to treat metastatic or recurrent laryngeal cancer.

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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.

Treatment for laryngeal cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

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 you go through treatment, you 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 condition has changed or if the cancer has recurred (come back).

About PDQ

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 adult laryngeal 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 Adult 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® Adult Treatment Editorial Board. PDQ Laryngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/patient/adult/laryngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389298]

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.

Incidence and Mortality

Estimated new cases and deaths from laryngeal cancer in the United States in 2025:[1]

Anatomy

The larynx is divided into the following three anatomical regions:

The supraglottic area is rich in lymphatic drainage. After penetrating the pre-epiglottic space and thyrohyoid membrane, lymphatic drainage is initially to the jugulodigastric and midjugular nodes. About 25% to 50% of patients present with involved lymph nodes. The precise figure depends on the T (tumor) stage. The true vocal cords are devoid of lymphatics. As a result, vocal cord cancer confined to the true cords rarely, if ever, presents with involved lymph nodes. Extension above or below the cords may, however, lead to lymph node involvement. Primary subglottic cancers, which are quite rare, drain through the cricothyroid and cricotracheal membranes to the pretracheal, paratracheal, and inferior jugular nodes, and occasionally to mediastinal nodes.[2]

Risk Factors

There is a clear association among smoking, excess alcohol ingestion, and the development of squamous cell cancers of the upper aerodigestive tract.[3] For smokers, the risk of laryngeal cancer decreases after they stop smoking but remains elevated, even years later, compared with that of nonsmokers.[4] If a patient who has had a single cancer continues to smoke and drink alcoholic beverages, the likelihood of a cure for the initial cancer, by any modality, is diminished, and the risk of second tumor is enhanced. Because of clinical problems related to smoking and alcohol use in this population, many patients die of intercurrent illness rather than the primary cancer.

Clinical Features

Supraglottic cancers typically present with sore throat, painful swallowing, referred ear pain, change in voice quality, or enlarged neck nodes. Early vocal cord cancers are usually detected because of hoarseness. By the time they are detected, cancers arising in the subglottic area commonly involve the vocal cords; thus, symptoms usually relate to contiguous spread.

Prognostic Factors

The most important adverse prognostic factors for laryngeal cancers include increasing T stage and N (regional lymph node) stage. Other prognostic factors may include sex, age, performance status, and a variety of pathological features of the tumor, including grade and depth of invasion.[5]

Prognosis for small laryngeal cancers that have not spread to lymph nodes is very good. Cure rates are 75% to 95% depending on the site, tumor bulk,[6] and degree of infiltration. Although most patients with early lesions can be cured by either radiation therapy or surgery, radiation therapy may be reasonable to preserve the voice, leaving surgery for salvage. Patients with a preradiation hemoglobin level higher than 13 g/dL have higher local control and survival rates than patients who are anemic.[7]

Locally advanced lesions are treated with combined modality treatment involving radiation and chemotherapy with or without surgery. The aim is laryngeal preservation in appropriately selected candidates.[8] Distant metastases are also common, even if the primary tumor is controlled.

Intermediate lesions have intermediate prognoses, depending on the site, T stage, N stage, and performance status. Therapy recommendations for patients with these lesions are based on a variety of complex anatomical, clinical, and social factors, which should be individualized and discussed in multidisciplinary consultation (surgery, radiation therapy, and dental and oral surgery) prior to prescribing therapy.

Follow-Up and Survivorship

Second primary tumors, often in the aerodigestive tract, have been reported in as many as 25% of patients whose initial lesion is controlled. A study has shown that daily treatment of these patients with moderate doses of isotretinoin (i.e., 13-cis-retinoic acid) for 1 year can significantly reduce the incidence of second tumors.[9] No survival advantage has been demonstrated, partially because of recurrence and death from the primary malignancy.

Patients treated for laryngeal cancers are at the highest risk of recurrence in the first 2 to 3 years. Recurrences after 5 years are rare and usually represent new primary malignancies. Close, regular follow-up is crucial to maximize the chance for salvage. Follow-up includes careful clinical examination and repetition of any abnormal staging study, along with attention to any treatment-related toxic effect or complication.

Definitions of TNM

The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define laryngeal cancer.[2]

AJCC Prognostic Stage Groups

Surgery and/or Radiation Therapy

Surgery and radiation therapy have been the standard treatments for laryngeal cancer. However, outcome data from randomized trials are limited. Studies have attempted to evaluate the use of surgery or radiation but have been underpowered.[1] Selection of primary surgery versus radiation therapy–based treatment should be made in a multidisciplinary setting with consideration of disease stage, comorbidities, and functional status, including voice and swallowing outcomes and lung capacity.

Small superficial cancers without laryngeal fixation or lymph node involvement are successfully treated by radiation therapy or surgery alone, including laser excision surgery. Radiation therapy may be selected to preserve the voice and to reserve surgery for salvaging failures. The radiation field and dose are determined by the location and size of the primary tumor. A variety of curative surgical procedures are also recommended for laryngeal cancers, some of which preserve vocal function. An appropriate surgical procedure must be considered for each patient, given the anatomical problem, performance status, and clinical expertise of the treatment team. Advanced laryngeal cancers are often treated by combining radiation with concurrent chemotherapy for larynx preservation and total laryngectomy for bulky T4 disease or salvage.[2–4]

Evaluation of treatment outcome can be reported in various ways: locoregional control, disease-free survival, determinate survival, and overall survival (OS) at 2 to 5 years. Preservation of voice is an important parameter to evaluate. Outcome should be reported after initial surgery, initial radiation, planned combined treatment, or surgical salvage of radiation failures. Primary source material should be consulted to review these differences.

A review of published clinical results of definitive radiation therapy for head and neck cancer suggests a significant loss of local control when radiation therapy was prolonged. Extending standard treatment schedules should be avoided whenever possible.[5,6]

Radiation therapy has not been directly compared with endolaryngeal surgery (with or without laser) for the treatment of patients with early-stage laryngeal cancer. The evidence is insufficient to show a clear difference in local control or OS for these two treatment options. Retrospective data suggest that, compared with surgery, radiation therapy might cause less perturbation of voice quality without a significant difference in patient perception.[7]

Concurrent Chemoradiation Therapy

Concurrent chemoradiation therapy is a standard treatment option for patients with locally advanced (stage III and stage IV) laryngeal cancer.

Evidence (concurrent chemoradiation therapy):

For more information about oral toxicities, see Oral Complications of Cancer Therapies.

Neoadjuvant Chemotherapy Followed by Concurrent Chemoradiation Therapy

In a meta-analysis of five randomized trials, a total of 1,022 patients with locally advanced head and neck squamous cell cancer were randomly assigned to receive either neoadjuvant chemotherapy with TPF (docetaxel, cisplatin, and fluorouracil [5-FU]) followed by concurrent chemoradiation therapy or concurrent chemoradiation therapy alone. The analysis failed to show an OS (HR, 1.01; 95% confidence limits [CLs], 0.84–1.21; P = .92) or PFS (HR, 0.91; 95% CLs, 0.75–1.1; P = .32) advantage for neoadjuvant chemotherapy using the TPF regimen over concurrent chemoradiation therapy alone.[11][Level of evidence A1]

Evidence (neoadjuvant chemotherapy followed by concurrent chemoradiation therapy):

Altered Fractionation Versus Standard Fractionation Radiation Therapy

Radiation therapy alone with altered fractionation may be used for patients with locally advanced laryngeal cancer who are not candidates for chemotherapy. Altered fractionation radiation therapy yields a higher locoregional control rate compared with standard fractionated radiation therapy for patients with stage III and stage IV head and neck cancer.

Evidence (altered fractionation vs. standard fractionation radiation therapy):

An additional late effect from radiation therapy is hypothyroidism, which occurs in 30% to 40% of patients who have received external-beam radiation therapy to the entire thyroid gland. Thyroid function testing of patients is a consideration before therapy and as part of posttreatment follow-up.[24,25]

Prospective data from two randomized controlled trials reported the incidence of hypothyroidism.[26]

For patients with well-lateralized oropharyngeal cancer, such as a T1 or T2 tonsil primary tumor with limited extension into the palate or tongue base and limited ipsilateral lymph node involvement without extracapsular extension, elective treatment to the ipsilateral lymph nodes results in only minimal risk of spread to the contralateral neck.[27] For T3 and T4 tumors that are midline or approach the midline, bilateral nodal treatment is a consideration. In addition to the cervical lymph node chain, retropharyngeal lymph nodes can also be encompassed in the elective nodal treatment.

Surgery Followed by Postoperative Radiation Therapy (PORT) With or Without Chemotherapy for Patients With Locally Advanced Disease

New surgical techniques for resection and reconstruction that provide access and functional preservation have extended the surgical options for patients with stage III or stage IV laryngeal cancer. Specific surgical procedures and their modifications are not described here because of the wide variety of surgical approaches, the variety of opinions about the role of modified neck dissections, and the multiple reconstructive techniques that may give the same results. This group of patients is managed by head and neck surgeons who are skilled in the multiple procedures available and are actively and frequently involved in the care of these patients.

Depending on pathological findings after primary surgery, PORT with or without chemotherapy is used in the adjuvant setting for the following histological findings:

The addition of chemotherapy to PORT for laryngeal cancer squamous cell carcinoma demonstrates a locoregional control and OS benefit compared with radiation therapy alone in patients who have high-risk pathological risk factors, extracapsular extension of a lymph node, or positive margins, based on a pooled analysis of the EORTC-22931 [NCT00002555] and RTOG-9501 [NCT00002670] studies.[28–31][Level of evidence A1]

For patients with intermediate pathological risk factors, the addition of cisplatin chemotherapy given concurrently with PORT is unclear. Intermediate pathological risk factors include:

The addition of cetuximab with radiation therapy in the postoperative setting for these intermediate pathological risk factors is being tested in a randomized trial (RTOG-0920 [NCT00956007]).

Supraglottis

Treatment options for stage I cancer of the supraglottis include:

Glottis

Treatment options for stage I cancer of the glottis include:

Subglottis

Treatment options for stage I cancer of the subglottis include:

Radiation therapy

Transoral CO₂ laser excision versus EBRT

Selection of treatment should include an evaluation of voice function and quality after treatment. Endoscopic CO₂ laser resections may also achieve similar results in terms of local control and function [8] compared with radiation therapy, although no randomized studies have been performed.[9]

Evidence (transoral CO₂ laser excision vs. EBRT):

Conventional radiation therapy versus hypofractionated radiation therapy

Conventional and hypofractionated radiation therapy regimens have been studied regarding radiation-dose fractionation for patients with early-stage larynx cancer.

Evidence (conventional radiation therapy vs. hypofractionated radiation therapy):

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Supraglottis

Treatment options for stage II cancer of the supraglottis include:

Radiation therapy should be preferred because of the good results, preservation of the voice, and the possibility of surgical salvage in patients whose disease recurs locally.

Glottis

Treatment options for stage II cancer of the glottis include:

Subglottis

Treatment options for stage II cancer of the subglottis include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Supraglottis

Treatment options for stage III cancer of the supraglottis include:

Glottis

Treatment options for stage III cancer of the glottis include:

Subglottis

Treatment options for stage III cancer of the subglottis include:

Role of Neck Dissection in the Post-Radiation Therapy Setting

A prospective randomized trial included 564 patients with head and neck cancer and N2 or N3 disease. Patients were assigned to undergo planned neck dissection or surveillance with positron emission tomography–computed tomography (PET-CT). With a median follow-up of 36 months, PET-CT resulted in fewer neck dissections compared with the surgical arm (54 vs. 221), with a 2-year survival rate of 84.9% versus 81.5%, respectively. The hazard ratio (HR)_death slightly favored PET-CT–guided surveillance and indicated noninferiority (upper boundary, 95% confidence interval for HR, <1.50; P = .004).[11][Level of evidence A1]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Supraglottis

Treatment options for stage IV cancer of the supraglottis include:

Glottis

Treatment options for stage IV cancer of the glottis include:

Subglottis

Treatment options for stage IV cancer of the subglottis include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Metastatic and Recurrent Laryngeal Cancer

Treatment options for metastatic and recurrent laryngeal cancer include:

For more information, see the Treatment Option Overview for Laryngeal Cancer section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult laryngeal cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Laryngeal Cancer Treatment are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Laryngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/hp/adult/laryngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389189]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. 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 Email Us.

Epidemiology

Cancer of the hypopharynx is uncommon; approximately 2,500 new cases are diagnosed in the United States each year.[1] The peak incidence of this cancer occurs in people aged 50 to 60 years.[2] Excessive alcohol and tobacco use are the primary risk factors for hypopharyngeal cancer.[3,4] In the United States, hypopharyngeal cancers are more common in men than in women.[5] In Europe and Asia, high incidences of pharyngeal cancers, namely, oropharyngeal and hypopharyngeal, have been found among men in France, in the counties of Bas-Rhin and Herault; Switzerland, in the section of Vaud; Spain, in the Basque Country region; Slovakia; Slovenia; and India, in the cities of Bombay and Madras.[6] This cancer is extremely rare in children.[7]

Upper hypopharyngeal cancers appear to be associated more with heavy drinking and smoking, whereas the lower hypopharyngeal, or postcricoid, cancers are more often associated with nutritional deficiencies.[1,8] Although earlier reports from northern Europe, particularly from Sweden, indicated a link between Plummer-Vinson syndrome, which consisted of sideropenic anemia and epithelial changes of the aerodigestive tract, and other nutritional deficiencies in women, current cases of hypopharyngeal cancer among women are more likely to be associated with excessive use of alcohol and tobacco, rather than with deficiency diseases.[2,9–11]

Anatomy

Anatomically, the hypopharynx extends from the plane of the hyoid bone above to the plane of the inferior border of the cricoid cartilage below. The hypopharynx is composed of the following three parts and does not include the larynx:

Clinical Features

The lymphatic drainage from the pharynx is into the jugulodigastric, jugulo-omohyoid, upper and middle deep cervical, and retropharyngeal nodes. In the United States and Canada, 65% to 85% of hypopharyngeal carcinomas involve the pyriform sinuses, 10% to 20% involve the posterior pharyngeal wall, and 5% to 15% involve the postcricoid area.[12] Pyriform sinus and postcricoid carcinomas are typically flat plaques with raised edges and superficial ulceration. In contrast, posterior hypopharyngeal wall tumors tend to be exophytic and are often large (i.e., 80% >5 cm) at presentation.[13] Hypopharyngeal carcinomas tend to spread within the mucosa, beneath intact epithelium, and are prone to skip metastasis and to resurface at various locations remote from the primary site.[1,13] Because of this fact and the abundant lymphatic network of the region, a localized hypopharyngeal tumor is the exception.[1]

Almost all hypopharyngeal cancers are mucosal squamous cell carcinomas (SCCs).[1] Multiple primary tumors are not uncommon. Approximately 25% of patients in a retrospective study of 150 cases were found to have second primary tumors.[14] Field cancerization may be responsible, in part, for the multiple, synchronous, primary malignant neoplasms that occur in patients with hypopharyngeal cancer.[1,14–16] The concept of field cancerization, originally described in 1953, proposes that tumors develop in a multifocal fashion within a field of tissue that has been chronically exposed to carcinogens.[17]

Clinically, cancers of the hypopharynx tend to be aggressive and demonstrate a natural history that is characterized by diffuse local spread, early metastasis, and a relatively high rate of distant spread. More than 50% of patients with hypopharyngeal cancer have clinically positive cervical nodes at the time of presentation. In 50% of these individuals, a neck mass is the presenting symptom.[2,18,19] In a retrospective study of 78 cases of hypopharyngeal cancer, other symptoms in addition to a neck mass (25.6%) included dysphagia (46.1%), odynophagia (44.8%), voice change (16.3%), and otalgia (14.2%).[2] A voice change resulting from pyriform sinus or postcricoid lesions is a late symptom that usually indicates invasion into the larynx or the recurrent laryngeal nerve.[1]

In a large retrospective study of patients with SCC of the larynx and hypopharynx, 87% of patients with pyriform sinus SCC were found to have stage III or stage IV disease; 82% of patients with SCC of the posterior pharyngeal wall were found to have stage III or stage IV disease.[20] As many as 17% of hypopharyngeal SCCs may be associated with distant metastases when clinically diagnosed.[20] This is quite different from the rate of distant metastasis detected at autopsy, which has been reported to be as high as 60%.[21] A relatively high incidence of delayed regional (i.e., 2 or more years after completion of primary therapy) and distant metastatic disease in hypopharyngeal SCC is related to the advanced stage of the disease at diagnosis. Almost 33% of pyriform sinus tumors may be associated with delayed regional metastases.[20]

The treatment of hypopharyngeal cancer is controversial, in part because of its low incidence and the inherent difficulty in conducting adequately powered, prospective, randomized clinical studies.[22] Therefore, it is difficult to define the ideal therapy for a specific site or stage of hypopharyngeal cancer. In general, both surgery and radiation therapy are the mainstays of most curative efforts. In recent years, chemotherapy has been added to the treatment strategies for selected advanced presentations of hypopharyngeal cancer.[23] In pyriform sinus cancer, neoadjuvant chemotherapy followed by radiation therapy may achieve larynx preservation without jeopardizing survival.[24]

Prognosis and Survival

Chronic pulmonary and hepatic diseases related to the excessive use of tobacco and alcohol are found in patients with hypopharyngeal cancer. Recognition of these comorbidities is essential when planning appropriate treatment.[1] The primary prognostic factors for hypopharyngeal SCC include:[1,25,26]

Factors that contribute to an overall poor prognosis with hypopharyngeal SCC include:

In many patients, a poor prognosis is related to poor overall health.[13] The most common cause of failure of treatment of the primary tumor is local and/or regional recurrence. Most treatment failures occur within the first 2 years following definitive therapy. The burden of lymph node metastases may yield information of prognostic value. In a retrospective study, a total volume of metastatic disease of more than 100 cm3 indicated a particularly poor prognosis.[25]

Risk Factors

In addition to the risk of delayed regional metastases, the risk of developing a second primary tumor in patients with tumors of the upper aerodigestive tract has been estimated to be 4% to 7% per year.[20,26–28] Because of these risks, surveillance of patients with hypopharyngeal cancer should be lifelong.

Histopathology

SCC of the hypopharynx has not been associated with any specific chromosomal or genetic abnormalities;[13] however, loss of chromosome 18 was observed in 57% of hypopharyngeal tumors in one study.[29] Several other studies have emphasized the importance of chromosome 11q13 amplification, which may be related to the presence of nodal metastases, greater local aggressiveness, and a higher incidence of tumor recurrence.[30–33]

American Joint Committee on Cancer (AJCC) Stage Groupings and TNM Definitions

The AJCC has designated staging by TNM (tumor, node, metastasis) classification to define hypopharyngeal cancer.[4]

Treatment Overview

Except for very early stage (T1) cancers of this region, treatment has primarily been surgery, usually followed with postoperative radiation therapy (PORT). Some early stage (T1 and T2), low-volume, exophytic pyriform sinus carcinomas have been successfully treated with radiation therapy alone.[3–5] Single-modality therapy of advanced-stage hypopharyngeal cancer, with either surgery or radiation therapy, has resulted in consistently poor survival.[6–8]

Combined-modality treatment should be considered for patients with stage III or stage IV disease.[4,6,9,10] When used with surgery, radiation therapy is typically administered postoperatively. In selected advanced cases, alternative strategies using neoadjuvant chemotherapy and radiation therapy may increase the chance for local control as much as resection and PORT.[4]

Fluorouracil Dosing

Treatment Options for Stage I Hypopharyngeal Cancer

Except for the very early T1 cancers of this region, treatment has been primarily surgery, usually followed with postoperative radiation therapy. Because these tumors are clinically silent until they reach advanced stages, it is very unusual to diagnose them at the T1 N0 stage. In most available retrospective reviews, T1 N0 cases represent only 1% to 2% of all patients seen. In the case of exophytic T1 N0 lesions, radiation therapy alone may be considered.[1,2]

Treatment options for stage I hypopharyngeal cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage II Hypopharyngeal Cancer

Treatment has been primarily surgery, which is usually followed with postoperative radiation therapy (PORT). Because these tumors are clinically silent until they reach advanced stages, it is very unusual to diagnose these tumors at the T2 N0 stage.

Treatment options for stage II hypopharyngeal cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage III Hypopharyngeal Cancer

The management of patients with stage III hypopharyngeal cancer is complex and requires multidisciplinary input to establish the optimal treatment regimen. New surgical techniques and reconstructions (using the gastric pull-up operation or free jejunal transfers) have greatly reduced the morbidity associated with resection of these tumors and have almost eliminated the need for multistage reconstructions. This has greatly aided the combined treatment regimens because these patients have a high likelihood of beginning postoperative radiation therapy (PORT) within 3 to 4 weeks following resection.

Details of surgical procedures and modifications of radiation fields or dosage schedules are not specifically designated here because of legitimate variations in techniques that, according to various retrospective data, give similar survival results in different treatment centers. This group of patients should be managed by surgeons and radiation oncologists who are skilled in the multiple procedures and techniques available, and who are actively and frequently involved in the care of these patients.

Treatment options for stage III hypopharyngeal cancer include:

For more information about treatment options for stage III hypopharyngeal cancer, see the Treatment Options for Unresectable Stage IV Hypopharyngeal Cancer section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Resectable Stage IV Hypopharyngeal Cancer

The management of patients with resectable hypopharyngeal cancer is complex and requires multidisciplinary input to establish the optimal treatment regimen. New surgical techniques and reconstructions using the gastric pull-up operation or free jejunal transfers have greatly reduced the morbidity associated with resection of these tumors and have almost eliminated the need for multistage reconstructions. This has greatly aided the combined treatment regimens because these patients have a high likelihood of beginning postoperative radiation therapy within 3 to 4 weeks following resection.

Details of surgical procedures and modifications of radiation fields or dosage schedules are not specifically designated here because of legitimate variations in techniques that, according to various retrospective data, give similar survival results in different treatment centers. This group of patients should be managed by surgeons and radiation oncologists who are skilled in the multiple procedures and techniques available, and who are actively and frequently involved in the care of these patients.

Treatment options for resectable stage IV hypopharyngeal cancer include:

Treatment Options for Unresectable Stage IV Hypopharyngeal Cancer

Treatment options for unresectable stage IV hypopharyngeal cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Metastatic and Recurrent Hypopharyngeal Cancer

Treatment options for metastatic and recurrent hypopharyngeal cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult hypopharyngeal cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Hypopharyngeal Cancer Treatment are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Hypopharyngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/hp/adult/hypopharyngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389199]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. 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 Email Us.

Hypopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the hypopharynx.

The hypopharynx is the bottom part of the pharynx. The pharynx is a hollow tube about 5 inches long that starts behind the nose, goes down the neck, and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus.

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Most hypopharyngeal cancers form in squamous cells, the thin, flat cells lining the inside of the hypopharynx. The hypopharynx has 3 different areas. Cancer may be found in 1 or more of these areas.

Hypopharyngeal cancer is a type of head and neck cancer.

Use of tobacco products and heavy drinking can affect the risk of developing hypopharyngeal cancer.

Anything that increases your risk 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 doctor if you think you may be at risk. Risk factors include:

Signs and symptoms of hypopharyngeal cancer include a sore throat and ear pain.

These and other signs and symptoms may be caused by hypopharyngeal cancer or by other conditions. Check with your doctor if you have:

Tests that examine the throat and neck are used to help diagnose hypopharyngeal cancer and find out whether the cancer has spread.

The following tests and procedures may be used:

Certain factors affect prognosis (chance of recovery) and treatment options.

Prognosis depends on:

Treatment options depend on:

Patients who have had hypopharyngeal cancer are at an increased risk of developing a second cancer in the head or neck. Frequent and careful follow-up is important.

After hypopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the hypopharynx or to other parts of the body.

The process used to find out if cancer has spread within the hypopharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of some of the tests and procedures used to diagnose hypopharyngeal cancer are often also used to stage the disease.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

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.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if hypopharyngeal cancer spreads to the lung, the cancer cells in the lung are actually hypopharyngeal cancer cells. The disease is metastatic hypopharyngeal cancer, not lung cancer.

The following stages are used for hypopharyngeal cancer:

The staging described below is only used for patients who have not had lymph nodes in the neck removed and checked for signs of cancer.

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After surgery, the stage of the cancer may change and more treatment may be needed.

If the cancer is removed by surgery, a pathologist will examine a sample of the cancer tissue under a microscope. Sometimes, the pathologist’s review results in a change to the stage of the cancer and more treatment is needed after surgery.

Hypopharyngeal cancer can recur (come back) after it has been treated.

The cancer may come back in the hypopharynx or in other parts of the body.

There are different types of treatment for patients with hypopharyngeal cancer.

Different types of treatment are available for patients with hypopharyngeal cancer. 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. 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.

The following types of treatment are used:

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.

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Radiation therapy may work better in patients who have stopped smoking before beginning treatment. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the body may be done before and after therapy to make sure the thyroid gland is working properly.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for hypopharyngeal cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

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 care may be needed.

As you go through treatment, you 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 condition has changed or if the cancer has recurred (come back).

For hypopharyngeal cancer, follow-up to check for recurrence should include careful head and neck exams once a month in the first year after treatment ends, every 2 months in the second year, every 3 months in the third year, and every 6 months thereafter.

About PDQ

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 hypopharyngeal 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 Adult 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® Adult Treatment Editorial Board. PDQ Hypopharyngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/patient/adult/hypopharyngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389254]

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.

Visual field exam

A visual field exam checks a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.

CT scan (CAT scan)

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.

Magnetic resonance imaging (MRI) with gadolinium

MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain and the spine. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Blood hormone studies

Blood hormone studies use a blood sample to measure the amounts of certain hormones released into the blood by organs and tissues in the body. If the amount of a hormone is higher or lower than normal, it can be a sign of disease in the organ or tissue that makes it. For craniopharyngioma, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). These hormones are made by the pituitary gland.

Biopsy

If the CT scan or MRI show there may be a brain tumor, your child will have a biopsy to remove a sample of the tumor.

Types of biopsy that may be used to take the sample of tissue include:

A pathologist views the tissue under a microscope to look for tumor cells. If they find tumor cells, the surgeon will remove as much tumor as safely possible during the same surgery.

Immunohistochemistry

Immunohistochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells or 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 to help tell one type of cancer from another type of cancer.

Who treats children with craniopharyngioma?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for childhood craniopharyngioma. The pediatric oncologist works with other health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. Other specialists may include:

Surgery

The type of surgery your child will have depends on the size of the tumor, where it is in the brain, and whether it has grown into nearby tissue in a finger-like way. It also depends on expected late effects that may occur after surgery.

The types of surgery that may be used to remove the tumor that can be seen with the eye include:

To help make a diagnosis, sometimes the surgeon will remove only part of the tumor. If a tumor is near the pituitary gland or hypothalamus, it will not be removed. Leaving the tumor helps reduce serious side effects from the surgery.

Sometimes, the surgeon will remove all of the tumor that they can see and no further treatment is needed. At other times, they may not be able to remove the tumor because it is growing into or pressing on nearby organs.

Surgery for cysts

If your child’s tumor is mostly a fluid-filled cyst, they may have surgery to drain it. Draining it lowers the pressure in the brain and relieves symptoms.

A surgery called a partial resection can be used to remove fluid from cystic craniopharyngiomas. Or a thin tube called a catheter can be inserted into the cyst, and a small container placed under the skin. The fluid drains into the container and is later removed.

Sometimes, after the cyst is drained, a drug is put through the catheter into the cyst. This causes the inside wall of the cyst to scar and stops the cyst from making fluid. Or it can slow down how long it takes for the fluid to build up again. Surgery to remove the tumor or radiation therapy may be done after the cyst is drained.

Radiation therapy

Radiation therapy uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. It is often given after surgery to kill any tumor that is left in the brain.

Both external radiation therapy and internal radiation therapy (also called brachytherapy) are used to treat craniopharyngiomas.

Radiation therapy to the brain can affect growth and development in young children, so ways of giving radiation therapy that have fewer side effects are often used. These include:

Learn more about Radiation Therapy to Treat Cancer.

Chemotherapy

Chemotherapy (also called chemo) uses drugs to stop the growth of tumor cells. Chemotherapy either kills the tumor cells or stops them from dividing.

Chemotherapy can be placed directly into a cavity, such as a cyst. This way of giving chemotherapy is intracavitary chemotherapy. Bleomycin is a type of chemotherapy that can be placed directly into a cystic craniopharyngioma.

Learn more about Chemotherapy to Treat Cancer.

Observation

Observation means that your child’s condition is closely watched without receiving treatment until symptoms appear or change.

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.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Treatment of newly diagnosed childhood craniopharyngioma

Treatment of newly diagnosed childhood craniopharyngioma may include:

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 progressive or recurrent childhood craniopharyngioma

Treatment options for progressive or recurrent childhood craniopharyngioma depend on the type of treatment that your child received when the tumor was first diagnosed and your child’s needs.

Treatment may include:

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.

About PDQ

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 craniopharyngioma. 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 Craniopharyngioma. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/brain/patient/child-cranio-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389237]

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.

Incidence and Mortality

Brain tumors account for 85% to 90% of all primary central nervous system (CNS) tumors.[1] Estimated new cases and deaths from brain tumors and other nervous system tumors in the United States in 2025:[2]

Data from the Surveillance, Epidemiology, and End Results (SEER) Program database for 2017 to 2021 indicated that the combined incidence of brain and other CNS tumors in the United States was 6.2 cases per 100,000 people per year. The mortality rate was 4.4 deaths per 100,000 people per year based on age-adjusted deaths from 2018 to 2022.[3] Worldwide, approximately 321,476 new cases of brain and other CNS tumors were diagnosed in the year 2022, with an estimated 248,305 deaths.[4]

In general, the incidence of primary CNS tumors is higher in White individuals than in Black individuals, and mortality is higher in men than in women.[3]

Primary brain tumors include the following in decreasing order of frequency:[1]

Primary spinal tumors include the following in decreasing order of frequency:

Primary brain tumors rarely spread to other areas of the body, but they can spread to other parts of the brain and to the spinal axis.

Anatomy

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Risk Factors

Few definitive observations have been made about environmental or occupational causes of primary CNS tumors.[1]

The following potential risk factors have been considered:

The following familial tumor syndromes and related chromosomal abnormalities are associated with CNS neoplasms:[6,7]

Clinical Features

The clinical presentation of various brain tumors is best appreciated by considering the relationship of signs and symptoms to anatomy.[1]

General signs and symptoms include:

Seizures are a presenting symptom in approximately 20% of patients with supratentorial brain tumors and may antedate the clinical diagnosis by months to years in patients with slow-growing tumors. Among all patients with brain tumors, 70% with primary parenchymal tumors and 40% with metastatic brain tumors develop seizures at some time during the clinical course.[8]

Diagnostic Evaluation

All brain tumors, whether primary, metastatic, malignant, or benign, must be differentiated from other space-occupying lesions that can have similar clinical presentations, such as abscesses, arteriovenous malformations, and infarctions.[9]

Prognostic Factors

Several genetic alterations have emerged as powerful prognostic factors in diffuse glioma (astrocytoma, oligodendroglioma, mixed glioma, and glioblastoma), and these alterations may guide patient management. Specific alterations include:

Other prognostic factors that confer poor prognosis include:[11,12]

An exploratory analysis of 318 patients with low-grade glioma treated with either radiation therapy alone or temozolomide chemotherapy alone reported the following results for patients with a combination of these prognostic factors:[11]

For more information, see the Treatment of Primary CNS Tumors by Tumor Type section.

Genomic Alterations

Alterations in the BRAF, IDH1, and IDH2 genes, and genomic 1p/19q codeletion, appear to be hallmark aberrations in particular glioma subtypes. Assessment for the presence of these variants aids diagnosis and prognosis and, with regard to 1p/19q codeletion, predicts for response to chemotherapy.

In pilocytic astrocytomas (WHO grade I), tandem duplication at 7q34 leading to a KIAA1549::BRAF gene fusion is found in approximately 70% of pilocytic astrocytomas.[6–8] Activating single nucleotide variants in BRAF (V600E) are found in an additional 5% to 9% of these tumors. Overall, RAF alterations occur in approximately 80% of pilocytic astrocytomas.

BRAF V600E variants are observed (in about 60%) of other benign gliomas, including pleomorphic xanthoastrocytoma and ganglioglioma, while BRAF tandem duplications are not found in these variant glioma tumors.[9–11]

Most WHO grade II and III diffuse gliomas (astrocytomas, oligodendrogliomas, and oligoastrocytomas) and 5% to 10% of glioblastomas (WHO grade IV) harbor single nucleotide variants in the R132 position of IDH1 or, rarely, the analogous codon in IDH2 (R172).[12–16] The presence of an IDH1 or IDH2 variant is a strong prognostic factor. Patients with these tumor variants have significantly longer survival independent of WHO grade or histological subtype.

Deletion of chromosomes 1p and 19q occurs through a translocation event [17] and is common in oligodendrogliomas. 1p/19q codeletion is a powerful prognostic factor and may predict for response to chemotherapy. For more information, see the Anaplastic oligodendrogliomas treatment section.

These genetic alterations have potential diagnostic utility. Presence of the IDH1 and IDH2 variants may distinguish diffuse gliomas from other gliomas, which often have BRAF genetic alterations, and nonneoplastic reactive astrocytosis.[18] Most (90%) IDH variants in gliomas result in an R132H substitution, which can be detected with a highly sensitive and specific monoclonal antibody. A rapid immunohistochemical analysis using the variant-specific IDH1 antibody can aid diagnostic analysis.[19]

Other CNS tumors are associated with characteristic patterns of altered oncogenes, altered tumor suppressor genes, and chromosomal abnormalities. Familial tumor syndromes with defined chromosomal abnormalities are associated with gliomas.

Primary CNS Tumors

This section discusses general treatment modalities for primary central nervous system (CNS) tumors. For a description of specific treatment options for each tumor type, see the Treatment of Primary CNS Tumors by Tumor Type section.

Radiation therapy and chemotherapy options vary according to histology and anatomical site of the CNS tumor. For glioblastoma, combined-modality therapy with resection, radiation, and chemotherapy is standard. Anaplastic astrocytomas, anaplastic oligodendrogliomas, and anaplastic oligoastrocytomas represent only a small proportion of CNS gliomas. Therefore, phase III randomized trials restricted to these tumor types are not generally practical. The natural histories of these tumors are variable, depending on histological and molecular factors; therefore, treatment guidelines for these tumors are evolving. Therapy involving surgically implanted carmustine-impregnated polymer wafers combined with postoperative external-beam radiation therapy (EBRT) may play a role in the treatment of high-grade (grades III and IV) gliomas in some patients.[1]

Treatment options for primary CNS tumors include:

Astrocytic Tumors Treatment

Oligodendroglial Tumors Treatment

Mixed Gliomas Treatment

Patients with mixed glial tumors, which include oligoastrocytoma (WHO grade II) and anaplastic oligoastrocytoma (WHO grade III), have highly variable prognoses based on their status of the IDH1 and IDH2 genes and 1p/19q chromosomes.[27–29] Therefore, the optimal treatment for these tumors as a group is uncertain. Often, they are treated similarly to astrocytic tumors because a subset of tumors may have outcomes similar to WHO grade III astrocytic or WHO grade IV glioblastoma tumors. Testing for these known, strong prognostic molecular markers should be performed, which may help to guide the assessment of risk and subsequent management.

Treatment options for mixed gliomas include:

For more information, see the Astrocytic Tumors Treatment section.

Ependymal Tumors Treatment

Ependymal tumors (WHO grade I) and ependymomas (WHO grade II)—i.e., subependymomas and myxopapillary ependymomas—are often curable.

Treatment options for grades I and II ependymal tumors include:

Patients with anaplastic ependymomas (WHO grade III) have variable prognoses that depend on the location and extent of disease. Frequently, but not invariably, patients with anaplastic ependymomas have worse prognoses than do those patients with lower-grade ependymal tumors.

Treatment options for anaplastic ependymomas include:

Embryonal Cell Tumors (Medulloblastomas) Treatment

Medulloblastoma occurs primarily in children but may also occur in adults.[31] For more information, see Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment.

Treatment options for medulloblastomas include:

Pineal Parenchymal Tumors Treatment

Pineocytomas (WHO grade II), pineoblastomas (WHO grade IV), and pineal parenchymal tumors of intermediate differentiation are diverse tumors that require special consideration. Pineocytomas are slow-growing tumors and prognosis varies.

Pineoblastomas grow more rapidly and patients with these tumors have worse prognoses. Pineal parenchymal tumors of intermediate differentiation have unpredictable growth and clinical behavior.

Treatment options for pineal parenchymal tumors include:

Meningeal Tumors Treatment

WHO grade I meningiomas are usually curable when they are resectable. With the increasing use of sensitive neuroimaging tools, there has been more detection of asymptomatic low-grade meningiomas. Most appear to show minimal growth and can often be safely observed while therapy is deferred until growth or the development of symptoms.[33,34]

Treatment options for meningeal tumors include:

The prognoses for patients with WHO grade II meningiomas (atypical, clear cell, and chordoid), WHO grade III meningiomas (anaplastic/malignant, rhabdoid, and papillary), and hemangiopericytomas are worse than the prognoses for patients with low-grade meningiomas because complete resections are less commonly feasible, and the proliferative capacity is greater.

Treatment options for grades II and III meningiomas and hemangiopericytomas include:

Germ Cell Tumors Treatment

The prognoses and treatment of patients with germ cell tumors—which include germinomas, embryonal carcinomas, choriocarcinomas, and teratomas—depend on tumor histology, tumor location, presence and levels of biological markers, and surgical resectability.

Treatment of Tumors of the Sellar Region

Craniopharyngiomas (WHO grade I) are often curable.

Treatment options for craniopharyngiomas include:

Treatment Options Under Clinical Evaluation for Primary CNS Tumors

Patients who have central nervous system (CNS) tumors that are either infrequently curable or unresectable should consider enrollment in clinical trials. Information about ongoing clinical trials is available from the NCI website.

Heavy-particle radiation, such as proton-beam therapy, carries the theoretical advantage of delivering high doses of ionizing radiation to the tumor bed while sparing surrounding brain tissue. The data are preliminary for this investigational technique and are not widely available.

Novel biological therapies under clinical evaluation for patients with CNS tumors include:[36]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

General Information About Metastatic Brain Tumors

Brain metastases outnumber primary neoplasms by at least 10 to 1, and they occur in 20% to 40% of cancer patients, with subsequent median survival generally less than 6 months.[1] The exact incidence is unknown because no national cancer registry documents brain metastases, but it has been estimated that 98,000 to 170,000 new cases are diagnosed in the United States each year.[2,3] This number may be increasing because of the capacity of magnetic resonance imaging (MRI) to detect small metastases and because of prolonged survival resulting from improved systemic therapy.[1,2]

The most common primary tumors with brain metastases and the percentage of patients affected are as follows:[1,2]

Eighty percent of brain metastases occur in the cerebral hemispheres, 15% occur in the cerebellum, and 5% occur in the brain stem.[2] Metastases to the brain are multiple in more than 70% of cases, but solitary metastases also occur.[1]

Brain involvement can occur with cancers of the nasopharyngeal region by direct extension along the cranial nerves or through the foramina at the base of the skull. Dural metastases may constitute as much as 9% of total brain metastases.

Clinical Features

The diagnosis of brain metastases in cancer patients is based on:

Patients may describe any of the following symptoms:

Often, family members or friends may notice the following changes:

Diagnostic Evaluation

A physical examination may show objective neurological findings or only minor cognitive changes. The presence of multiple lesions and a high predilection of primary tumor metastasis may be sufficient to make the diagnosis of brain metastasis.

A lesion in the brain should not be assumed to be a metastasis just because a patient has had a previous cancer; such an assumption could result in overlooking appropriate treatment of a curable tumor.

Treatment of Metastatic Brain Tumors

The optimal therapy for patients with brain metastases continues to evolve.[1,2,5] The following treatments have been used in the management of metastatic brain tumors:

Because most cases of brain metastases involve multiple metastases, a mainstay of therapy has historically been whole-brain radiation therapy (WBRT). However, stereotactic radiosurgery has become increasingly common. The role of radiosurgery continues to be defined. Stereotactic radiosurgery in combination with WBRT has been assessed.

Surgery is indicated to obtain tissue from a metastasis with an unknown primary tumor or to decompress a symptomatic dominant lesion that is causing significant mass effect.

Chemotherapy is usually not the primary therapy for most patients; however, it may have a role in the treatment of patients with brain metastases from chemosensitive tumors and can even be curative when combined with radiation for metastatic testicular germ cell tumors.[1,6] Intrathecal chemotherapy is also used for meningeal spread of metastatic tumors.

Leptomeningeal Carcinomatosis (LC)

LC occurs in about 5% of all cancer patients. The most common types of cancer to spread to the leptomeninges are:

Diagnosis includes a combination of neurospinal axis imaging and cerebrospinal fluid (CSF) cytology. Median OS is in the range of 10 to 12 weeks.

The management of LC includes:

In a series of 149 patients with metastatic non-small cell lung carcinoma, cytologically proven LC, poor performance status, high protein level in the CSF, and a high initial CSF white blood cell count were significant poor prognostic factors for survival.[19] Patients received active treatment, including intrathecal chemotherapy, WBRT, or epidermal growth factor receptor-tyrosine kinase inhibitors, or underwent a ventriculoperitoneal shunt procedure.

In a retrospective series of 38 patients with metastatic breast cancer and LC, the proportion of LC cases varied by breast cancer subtype:[20]

Patients with triple-negative breast cancer had a shorter interval between metastatic breast cancer diagnosis and the development of LC. Median survival did not differ across breast cancer subtypes. Consideration of intrathecal administration of trastuzumab in patients with HER2-positive LC has also been described in case reports.[21]

Chemotherapy

Antiangiogenesis Therapy

In 2009, the U.S. Food and Drug Administration (FDA) granted accelerated approval of bevacizumab monotherapy for patients with progressive glioblastoma. The indication was granted under the FDA’s accelerated approval program that permits the use of certain surrogate end points or an effect on a clinical end point other than survival or irreversible morbidity as bases for approvals of products intended for serious or life-threatening illnesses or conditions.

The approval was based on the demonstration of improved objective response rates observed in two historically controlled, single-arm, or noncomparative phase II trials.[3,4][Level of evidence C3] Based on these data and the FDA approval, bevacizumab monotherapy has become standard therapy for recurrent glioblastoma.

Evidence (antiangiogenesis therapy):

No data are available from prospective randomized controlled trials demonstrating improvement in health outcomes, such as disease-related symptoms or increased survival with the use of bevacizumab to treat glioblastoma.

Radiation Therapy

Because there are no randomized trials, the role of repeat radiation after disease progression or the development of radiation-induced cancers is also ill defined. Interpretation is difficult because the literature is limited to small retrospective case series.[5] The decision must be made carefully because of the risk of neurocognitive deficits and radiation necrosis.

Surgery

Re-resection of recurrent CNS tumors is an option for some patients. However, most patients do not qualify because of a deteriorating condition or technically inoperable tumors. The evidence is limited to noncontrolled studies and case series of patients who are healthy enough and have tumors that are small enough to technically debulk. The impact on survival of reoperation versus patient selection is not known.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult central nervous system tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Central Nervous System Tumors Treatment are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Central Nervous System Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/brain/hp/adult-brain-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389419]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. 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 Email Us.

Blood chemistry study

Blood chemistry study uses a blood sample to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual amount of a substance can be a sign of disease.

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. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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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.

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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. 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.

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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.

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24-hour urine test

A 24-hour urine test collects urine for 24 hours to measure the amounts of certain substances, such as hormones. An unusual amount of a substance can be a sign of disease in the organ or tissue that makes it. The urine sample is checked to see if it contains 5-HIAA (a breakdown product of the hormone serotonin which may be made by neuroendocrine tumors). This test is used to help diagnose carcinoid syndrome.

Somatostatin receptor scintigraphy

Somatostatin receptor scintigraphy is 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.

Who treats children with gastrointestinal neuroendocrine tumor?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of gastrointestinal neuroendocrine tumor. 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:

Treatment options

There are different types of treatment for children and adolescents with a gastrointestinal neuroendocrine tumor. 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.

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, visit our booklet, Children with Cancer: A Guide for Parents.

Types of treatment your child might have include:

Treatment of childhood gastrointestinal neuroendocrine tumor

Treatment of neuroendocrine tumor in the appendix in children is surgery to remove the appendix.

Treatment of neuroendocrine tumor in the large intestine, pancreas, or stomach is usually surgery.

Treatment of neuroendocrine tumor that cannot be removed by surgery, multiple tumors, or tumors that have spread may include:

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.

About PDQ

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 gastrointestinal neuroendocrine 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 Gastrointestinal Neuroendocrine Tumors. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/gi-neuroendocrine-tumors/patient/child-gi-neuroendocrine-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.

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.

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The adrenal cortex makes important hormones that:

Adrenocortical carcinoma is also called cancer of the adrenal cortex. A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make more hormones than normal). Most adrenocortical tumors are functioning. The hormones made by functioning tumors may cause certain signs or symptoms of disease.

The adrenal medulla makes hormones that help the body react to stress. Cancer that forms in the adrenal medulla is called pheochromocytoma and is not discussed in this summary. Learn more about Pheochromocytoma and Paraganglioma.

Adrenocortical carcinoma and pheochromocytoma can occur in both adults and children. Treatment for children, however, is different than treatment for adults. Learn more about Childhood Adrenocortical Carcinoma Treatment and Childhood Pheochromocytoma and Paraganglioma Treatment.

Having certain genetic conditions increases the risk of adrenocortical carcinoma.

Anything that increases a person’s risk of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop adrenocortical carcinoma, and it will develop in some people who don’t have any known risk factors. Talk with your doctor if you think you may be at risk.

Risk factors for adrenocortical carcinoma include having the following hereditary diseases:

Symptoms of adrenocortical carcinoma include pain in the abdomen.

These and other signs and symptoms may be caused by adrenocortical carcinoma:

A nonfunctioning adrenocortical tumor may not cause signs or symptoms in the early stages.

A functioning adrenocortical tumor makes too much of one of the following hormones:

Too much cortisol may cause:

Too much aldosterone may cause:

Too much testosterone (in women) may cause:

Men who make too much testosterone do not usually have signs or symptoms.

Too much estrogen (in women) may cause:

Too much estrogen (in men) may cause:

These and other signs and symptoms may be caused by adrenocortical carcinoma or by other conditions. Check with your doctor if you have any of these problems.

Imaging studies and tests that examine the blood and urine are used to diagnose adrenocortical carcinoma.

The tests and procedures used to diagnose adrenocortical carcinoma depend on the patient’s signs and symptoms. In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

Certain factors affect the prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on:

Adrenocortical carcinoma may be cured if treated at an early stage.

After adrenocortical carcinoma has been diagnosed, tests are done to find out if cancer cells have spread within the adrenal gland or to other parts of the body.

The process used to find out if cancer has spread within the adrenal gland or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

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.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if adrenocortical carcinoma spreads to the lung, the cancer cells in the lung are actually adrenocortical carcinoma cells. The disease is metastatic adrenocortical carcinoma, not lung cancer.

The following stages are used for adrenocortical carcinoma:

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Adrenocortical carcinoma can recur (come back) after it has been treated.

The cancer may come back in the adrenal cortex or in other parts of the body.

There are different types of treatment for patients with adrenocortical carcinoma.

Different types of treatments are available for patients 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. 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.

The following types of treatment are used:

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.

Treatment for adrenocortical carcinoma may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

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 care may be needed.

As you go through treatment, you 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 condition has changed or if the cancer has recurred (come back).

About PDQ

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 adult 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 Adult 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® Adult Treatment Editorial Board. PDQ Adrenocortical Carcinoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/adrenocortical/patient/adrenocortical-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389225]

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.

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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.