Lymphedema occurs when disruption of normal lymphatic drainage leads to accumulation of protein-rich lymph fluid in the interstitial space. Cancer survivors who experience lymphedema report poor physical functioning, impaired ability to engage in normal activities of daily living, and increased psychological distress.[1–5]

Estimates of the prevalence of lymphedema vary widely due to differences in the type of cancer, measurement methods, diagnostic criteria, and timing of evaluations relative to cancer diagnosis and treatment. In a survey conducted in 2006 and 2010, 6,593 cancer survivors were asked to identify ongoing concerns. Approximately 20% of respondents reported concerns related to lymphedema. Of these individuals, 50% to 60% reported receiving care for lymphedema.[6] These results align reasonably well with a survey study of women survivors of ovarian, endometrial, and colorectal cancers, who met criteria for lymphedema based on a validated survey that demonstrated a point prevalence of 37%, 33%, and 31%, respectively.[3] Similarly, a randomized intervention study in women with breast cancer demonstrated, by limb volume measurements or physician diagnosis, that 42% of subjects had lymphedema at 18 months after surgery.[7][Level of evidence: I]

Lymphedema is a common delayed effect of cancer treatment that negatively impacts survivors’ quality of life. This summary reviews the anatomy of the lymphatic system, the pathophysiology of lymphedema secondary to cancer, and epidemiology. The summary also provides clinicians with information related to risk factors, diagnosis, prevention, and treatment. The summary does not deal with congenital lymphedema or lymphedema not related to cancer.

In this summary, unless otherwise stated, evidence and practice issues as they relate to adults are discussed. The evidence and application to practice related to children may differ significantly from information related to adults. When specific information about the care of children is available, it is summarized under its own heading.

Anatomy of the Lymphatic System

The human lymphatic system generally includes superficial or primary lymphatic vessels that form a complex dermal network of capillary-like channels. Primary lymphatic vessels lack muscular walls and do not have valves. They drain into larger, secondary lymphatic vessels located in the subdermal space. Secondary lymphatic vessels run parallel to the superficial veins and drain into deeper lymphatic vessels located in the subcutaneous fat adjacent to the fascia. Unlike the primary vessels, the secondary and deeper lymphatic vessels have muscular walls and numerous valves to accomplish active and unidirectional lymphatic flow.

An intramuscular system of lymphatic vessels that parallels the deep arteries and drains the muscular compartment, joints, and synovium also exists. The superficial and deep lymphatic systems probably function independently, except in abnormal states, although there is evidence that they communicate near lymph nodes.[8] Lymph drains from the lower limbs into the lumbar lymphatic trunk. The lumbar lymphatic trunk joins the intestinal lymphatic trunk and cisterna chyli to form the thoracic duct, which empties into the left subclavian vein. The lymphatic vessels of the left arm drain into the left subclavian lymphatic trunk and then into the left subclavian vein. The lymphatic vessels of the right arm drain into the right subclavian lymphatic trunk and then into the right subclavian vein.

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Pathophysiology of Lymphedema

Body fluids can be discussed in terms of their composition and the specific fluid compartment where they are located. Intracellular fluid includes all fluid enclosed by the plasma membranes of cells. Extracellular fluid (ECF) surrounds all cells in the body. ECF has two primary constituents: intravascular plasma and the interstitial fluid that surrounds all cells not in the plasma. Lymphedema is the abnormal accumulation of protein-rich fluid in the interstitial space that is accompanied by inflammation and, eventually, fibrosis.

The formation of interstitial fluid comes from the movement of intravascular fluid across the capillary membranes due to arteriolar blood pressure. Much of the interstitial fluid returns to the intravascular fluid via the postcapillary venules. The dynamics of fluid production are influenced by arterial and venous hydrostatic pressures, tissue pressure, oncotic pressures of the intravascular and interstitial fluid, and membrane permeability. Normally, the dynamics favor a net gain of interstitial fluid, with the excess removed via lymphatic channels. Because lymphatic vessels often lack a basement membrane, they can resorb molecules too large for venous uptake as well. In short, the lymphatic system controls the pressure, volume, and composition of the interstitial fluid.

Lymphatic obstruction leads to increased interstitial fluid, which often contains large proteins and cellular debris. Through mechanisms not fully understood, the increased interstitial fluid induces inflammation, destruction or sclerosis of the lymphatic vessels, fibrosis, and, ultimately, adipose tissue hypertrophy.

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Epidemiology and Risk Factors

Accurate estimates of the incidence and prevalence of lymphedema are difficult to provide, due in part to differences in the definition of lymphedema (e.g., patient self-reports vs. objective volume measurements) and the timing of assessment for lymphedema relative to cancer treatment. Other factors are differences in surgical techniques related to the type of lymph node dissection or the total dose, fractions, and field of radiation administered.

Common risk factors for developing lymphedema include the following:

Breast Cancer

A systematic review found the prevalence of lymphedema to be 21.4% (14.9%–29.8%) in patients with breast cancer.[1] The incidence increased for up to 2 years after breast cancer diagnosis or surgery, and it was higher in women who underwent axillary lymph node dissection versus sentinel lymph node biopsy (19.9% vs. 5.6%). As a result, omission of axillary dissection in women with an involved sentinel lymph node is now an accepted practice. This practice is a result of a phase III randomized study (ACOSOG-Z0011) that showed no difference in overall survival in women who did not undergo a complete axillary dissection, compared with those who did.[2] Additional risk factors for lymphedema development included greater number of lymph nodes dissected, having a mastectomy, and overweight or obesity.[1,3] In a prospective study of neoadjuvant chemotherapy followed by axillary lymph node dissection (ACOSOG-Z1071), the incidence of lymphedema after a median follow-up of 3 years was 37.8% (95% confidence interval [CI], 33.1%–43.2%). Increasing body mass index (BMI) (hazard ratio [HR], 1.04; 95% CI, 1.01–1.06), duration of neoadjuvant chemotherapy (HR, 1.48; 95% CI, 1.01–2.17), number of lymph nodes removed, and number of involved lymph nodes were associated with lymphedema symptoms.[4]

Several risk factors for breast cancer–related lymphedema (BCRL) were demonstrated in a study using data from a 2-year, prospective observational study of 304 patients with breast cancer who had axillary lymph node dissection and radiation therapy. The cumulative incidence of lymphedema was measured by a more than 10% increase in arm volume, and univariate and multivariable analyses were performed. On multivariable analysis, Black race and Hispanic ethnicity (odds ratio [OR], 3.88; 95% CI, 2.14–7.08, and OR, 3.01; 95% CI, 1.10–7.62, respectively; P < .001 for each), receipt of neoadjuvant chemotherapy (OR, 2.10; 95% CI, 1.16–3.95; P = .01), older age (OR, 1.04; 95% CI, 1.02–1.07 per 1-year increase; P = .001), and a longer follow-up interval (OR, 1.57; 95% CI, 1.30–1.90 per 6-month increase; P < .001) were independently associated with an increased risk.[5][Level of evidence: II]

Another study examined risk factors for BCRL related to treatment, comorbidities, and lifestyle in 918 women enrolled in a Prospective Surveillance and Early Intervention (PSEI) trial. Women were randomly assigned to either bioimpedance spectroscopy (BIS) or tape measurement (TM).[6] In a secondary analysis, risk factors were used to test for factor associations with outcomes (no lymphedema, subclinical lymphedema, progression to chronic lymphedema after intervention, progression to chronic lymphedema without intervention). Factors associated with BCRL risk included axillary lymph node dissection (P < .001), taxane-based chemotherapy (P < .001), regional nodal irradiation (P ≤ .001), BMI greater than 30 (P = .002), and rurality (P = .037).[6]

Gynecological Cancers

A cohort study supports the evidence that a significant proportion of women experience lower-limb lymphedema after treatment of gynecological cancer or colorectal cancer. The highest prevalence (36.5%) among ovarian cancer survivors, followed by endometrial cancer survivors (32.5%) and colorectal cancer survivors (31.4%).[7]

In one study, 802 of 1,774 women diagnosed with a gynecological cancer between 1999 and 2004 responded to a survey about lymphedema.[8] Twenty-five percent of the respondents reported lower-extremity edema; 10% had been diagnosed with lymphedema. Most respondents (75%) had been diagnosed with these conditions within the first year of a cancer diagnosis. Women with vulvar cancer were most likely to have symptoms (36%). Lymph node dissection increased the risk of symptoms in women with cervical cancer but not uterine cancer. The range of symptoms included heavy legs, pain, and skin tightness. Standing all day, long-distance travel, and hot weather were precipitating conditions. The most common treatments were compression garments, massage, and lymphatic exercises. The findings related to high prevalence in patients with vulvar cancer and occurrence of symptoms within the first year have been verified.[9]

Serial limb volume measurements were obtained from a cohort of women who underwent a lymph node dissection for vulvar (n = 42), endometrial (n = 734), or cervical (n = 138) cancer 4 to 6 weeks after surgery and then every 3 months.[10] The incidence of an increase in limb volume of more than 10% was 43% for vulvar cancer, 34% for endometrial cancer, and 33% for cervical cancer. The incidence of severe lymphedema (>40% increase in volume) was less than 2% in all cohorts. The peak incidence was at the 4- to 6-week time point, but new cases were identified at all time points. The risk-factor analysis identified a reduced risk in women older than 65 years and a higher risk in women who had more than eight lymph nodes removed in the endometrial cohort.

Head and Neck Cancer

Patients with head and neck cancer are susceptible to external and internal lymphedema. External lymphedema typically presents with submental edema or lower neck swelling. Internal lymphedema is more widely distributed in the anatomical regions of the oropharynx. In a small cross-sectional study with video-assisted examinations, 59 of 61 patients had some degree of lymphedema.[11][Level of evidence: II] Sixty-one percent of the patients had only internal lymphedema, 35% had internal and external lymphedema, and 4% had only external lymphedema. Postoperative radiation therapy was a risk factor for combined lymphedema. Chemotherapy was a risk factor for patients with internal lymphedema only.

Melanoma

One single-center, cross-sectional study reported on lymphedema after either sentinel lymph node biopsy or lymph node dissection in 435 patients who were treated for melanoma between 1997 and 2015.[12] The authors reported a lymphedema prevalence of 25%. Forty-eight patients (44%) had International Society of Lymphology (ISL) stage I lymphedema (pitting edema), and 61 patients (56%) had ISL stage II or III lymphedema. Multivariate analyses identified as potential predictive factors the primary site of disease on the affected limb, inguinal surgery, and persistent pain at the site of lymph node surgery. Limb cellulitis was a risk factor for ISL stage II and III lymphedema. The same investigators also reported on health-related quality of life in an earlier publication. In another smaller, single-institution, retrospective study of 66 patients who underwent therapeutic nodal dissection, the rate of permanent lymphedema for inguinal node dissection was 38%, compared with 12% for axillary node dissection.[13] Another potentially relevant variable is the type of dissection. A 2017 systematic review did not find an appreciable difference between the rate of lymphedema after therapeutic lymphadenectomy and completion of lymph node sampling after a positive sentinel lymph node biopsy.[14] In both instances, the rate was around 20%.

Prostate Cancer

There are few studies of lymphedema after prostate cancer therapy. A small cross-sectional survey of men who underwent radical prostatectomy reported that 19 of 54 respondents (35.2%) had bilateral lower-extremity lymphedema.[15][Level of evidence: II] Of note, 25 respondents reported that they had received manual lymphatic drainage therapy. Men who did not experience regression experienced more distress related to physical and mental functioning than those who did. An elevated BMI and poor general health were risk factors for lymphedema.

Sarcoma

One study measured patient demographics, surgical outcomes data, functional outcomes, and lymphedema severity with a validated scale for 289 patients who underwent limb preservation surgery of an extremity sarcoma between 2000 and 2007.[16] The mean time from surgery was 35 months (range, 12–60 months). Eighty-three patients had some degree of lymphedema, including 58 with mild but definite swelling, 22 with moderate swelling, and 3 with considerable swelling. No patients had grade 4 or very severe swelling with shiny skin and skin cracking. Univariate analysis demonstrated that radiation therapy, tumor size, and tumor depth correlated with severity. The location of the sarcoma (upper or lower extremity), lymph node dissection (yes or no), and BMI did not correlate with severity. Multivariable analysis demonstrated that tumor size was the only independent predictor.

Signs, Symptoms, and Physical Examination

Lymphedema is typically evident by clinical findings such as unilateral, nonpitting edema, usually with involvement of the digits, in a patient with known risk factors (e.g., a breast cancer patient with previous axillary dissection). Other causes of limb swelling, including deep venous thrombosis, malignancy, and infection, should be considered in the differential diagnosis and excluded with appropriate studies, if indicated.

Lymphedema in patients with head and neck cancer can present slightly differently. External lymphedema does show swelling in the head and neck area, but internal lymphedema does not. Instead, patients with lymphedema related to internal head and neck cancer can present with complaints of voice changes, dysphagia, and possible difficulty breathing.

Diagnostic Testing

Nonsurgical Options

Surgical Options

The surgical options for the treatment of lymphedema include lymphatico-venous anastomoses (LVA), vascularized lymph node transplantation (VLNT), and reduction of excess tissue volume by excision of liposuction. Several informative reviews describe the surgical decision making involved in selecting patients and the type of operation.[14]

There are limited data to guide the choice between liposuction and microsurgical techniques, and some investigators propose a combined approach.[15] The choice of microsurgical techniques may be aided by imaging and clinical grading of lymphedema severity. One proposal suggests that patients are candidates for LVA if they have partial obstruction seen on lymphoscintigraphy and grade 1 or 2 lymphedema with patent lymphatic ducts observed on indocyanine green lymphography . On the other hand, VLNT may be better for patients exhibiting a total obstruction seen on lymphoscintigraphy and grade 3 or 4 lymphedema without patent lymphatic ducts observed on indocyanine green lymphography.[16]

Breast Cancer: Prevention of Lymphedema

Breast Cancer: Treatment of Lymphedema

Cervical Cancer: Prevention of Lymphedema

One study enrolled 120 women with cervical cancer who underwent a laparoscopic radical hysterectomy with pelvic lymphadenectomy. Participants were randomly assigned to an education-alone intervention or a CDT intervention.[11][Level of evidence: I] The CDT consisted of training in manual lymphatic drainage, followed by self-administered manual lymphatic drainage at home, compression hosiery, and an aerobic exercise program. The interventions were initiated 7 to 10 days after surgery. Additional eligibility criteria included more than 20 lymph nodes removed or anticipated radiation therapy (both of which are risk factors for lower-extremity lymphedema after surgery for cervical cancer). The primary outcome was limb volume calculated from multiple circumference measurements. Secondary outcomes included patients’ self-reported symptoms related to lymphedema. After a follow-up of 1 year, 20 of 58 patients (24%) in the control arm and 8 of 59 (14%) in the CDT arm developed lymphedema (P = .008). The excess volume was less in the experimental arm as well. However, there were no differences in patient-reported symptoms or severity grading of the lymphedema. These promising results were supported by a smaller pilot study[12] and a retrospective review of a single-institution experience with women who developed lymphedema after treatment of a variety of gynecological cancers.[13]

Head and Neck Cancer: Treatment of Lymphedema

Sarcoma of the Extremity: Prevention of Lymphedema

One study compared the incidence of lymphedema in a cohort of eight patients with thigh sarcoma, who had lymphatico-venous anastomoses performed in combination with resection of thigh soft-tissue tumors, with a historical cohort of 20 patients.[18] Only one of eight patients experienced lymphedema, compared with nine patients in the historical cohort. Patient self-reported symptoms were uncommon in the eight patients.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the diagnosis and treatment of lymphedema. 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 Supportive and Palliative Care 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 Lymphedema 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 Supportive and Palliative Care 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® Supportive and Palliative Care Editorial Board. PDQ Lymphedema. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /side-effects/lymphedema/lymphedema-hp-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389244]

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Introduction

Dyspnea is an uncomfortable awareness of breathing. It is a subjective experience involving many factors that affect the quality and intensity of its perception. Patients with comparable degrees of functional lung impairment and disease burden may describe varying intensities of dyspnea, using various words and phrases such as “tightness” and “suffocating.”[1]

Reports on the frequency of dyspnea also vary, depending on the setting and the extent of disease.[2] In one study, 49% of a general cancer population reported breathlessness, and 20% rated their breathlessness as moderate to severe.[3] Patients with advanced cancer experience dyspnea more frequently and more intensely than patients with limited disease. One study found that 75 of 135 patients with advanced cancer reporting to an outpatient palliative care clinic had moderate-to-severe dyspnea.[4] In addition, 60% of 289 patients with lung cancer reported breathlessness at presentation.[5] Results of a large study showed that 70% of patients suffered from dyspnea in the last 6 weeks of life.[6] About one-third of patients who could report the intensity of their dyspnea rated it as moderate to severe. Another study revealed that one-half of patients with advanced cancer scored their dyspnea as moderate to severe.[7]

Pathophysiology and Etiology

The pathophysiological mechanisms of breathlessness are numerous and complex.[8] Peripheral and central mechanisms, as well as mechanical and chemical pathways, are involved with a variety of sensory afferent sources.[9–11]

The qualities of dyspnea can be seen as work/effort, tightness, and air hunger. The experience of excess work/effort is caused by sensory-perceptual mechanisms similar to those involved in muscles exercising. Tightness is caused by stimulation of airway receptors with bronchoconstriction. The intensity of air hunger and unsatisfied inspiration is caused by the following:[12]

Dyspnea in patients with advanced cancer has many possible direct causes, and categorizing them can assist in the etiologic work-up. One approach is to divide direct causes into the following groups:

One study found that in patients experiencing dyspnea from advanced cancer, a median of five different abnormalities could have contributed to their shortness of breath.[7] Spirometry was abnormal in 93% of 100 patients examined, with 5% having obstructive patterns, 41% restrictive patterns, and 47% mixed patterns. Also, 49% of patients had lung cancer, 91% had abnormal chest radiographs, and 65% had parenchymal or pleural involvement. These results show that a subset of patients experience shortness of breath without any apparent lung involvement. The potentially correctable causes of dyspnea included:

No significant association between the type of respiratory impairment and the degree of dyspnea was found. Most of these patients were current or former smokers and had a significant lowering of their maximum inspiratory pressures, suggesting severe respiratory muscle dysfunction.[7] This finding was duplicated in another study.[4] Of patients admitted to hospice care, 34% had histories of cardiac disease, and 24% had histories of respiratory disease.[6] Only 39% of terminally ill patients who reported dyspnea had lung or pleural involvement. The etiology of dyspnea could not be clearly identified in approximately one-quarter of patients. Another study found that 49% of lung cancer patients presented with airflow obstruction.[7,15]

Respiratory muscle dysfunction is an underrecognized factor contributing to dyspnea. Causes of respiratory muscle dysfunction include:[16]

Poor oxygenation, muscle fatigue, abnormal cortisol and catecholamine levels, and circulating cytokines are also implicated.[16]

Although anxiety is commonly thought to be associated with breathlessness, researchers have found that anxiety and shortness of breath do not invariably go together.[7] One study demonstrated that the involvement of the lungs by cancer, anxiety, and poor maximal inspiratory pressures were correlates of the intensity of dyspnea in patients with advanced cancer.[4]

Assessment

The multidimensional nature of dyspnea must be noted in the complicated assessment of this symptom. Patient-reported outcome is the gold standard for assessment of dyspnea. There is no consensus on the best instrument for assessing dyspnea, but some of the tools used include:

These tools are limited, however, because they are unidimensional and do not account for the relative contribution of different factors to a patient’s perception of breathlessness. Assessment should include the impact of dyspnea on the patient’s functional status and recognition of the dynamic component of dyspnea—namely, exertional dyspnea.

Objective signs, such as tachypnea or the use of accessory breathing muscles, frequently do not match a patient’s perception of dyspnea and the degree of functional impairment it causes. Numerous factors, including psychosocial issues, may affect a patient’s experience of dyspnea. Pulmonary function tests, with few exceptions, play a limited role in the assessment of this syndrome.

A comprehensive history and examination are essential to an accurate assessment of dyspnea.[8,17] The temporal onset, qualities of the symptom, associated symptoms, precipitating and relieving events or activities, and responses to medications should be reviewed. Sudden onset may herald a pulmonary embolism or infection, whereas gradual onset may suggest development of a pleural effusion. A history of obstructive airways or cardiac disease can shed some light on possible underlying causes. Investigations such as measuring oxygen saturation can be useful in determining whether a patient is hypoxic. In the setting of advanced, incurable cancer, arterial blood gasses play a limited role.

In the study looking at checkpoint inhibitor immunotherapy–related pneumonitis, one-third of patients were asymptomatic. However, the most common presenting symptoms were dyspnea (53%) and cough (35%).[13]

Melanoma and non-small cell lung cancer were the most common cancers treated in this study. Interestingly, the duration of treatment before the onset of pneumonitis was quite variable, with a median time to onset of 2.8 months (range, 9 days–19 months). In addition, pneumonitis seemed to occur earlier in patients who received combination therapy than in those who received monotherapy (median, 2.7 months vs. 4.6 months).[13]

Diagnostic tests that may help to determine the etiology of dyspnea include the following:[7]

Maximal inspiratory pressure (MIP) measurements may be helpful, particularly if no apparent cause can be found. MIP is a reliable functional test of the strength of the diaphragm and other respiratory muscles. Functional assessments such as the 6-minute walk test and exercise ergometry may also provide valuable information about the severity and impact of dyspnea.[21,22]

Management of Dyspnea

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.

Significance

Malignant pleural effusions are a common complication of malignancy, and malignancy is a common cause of pleural effusions in general. Malignancy causes roughly 40% of symptomatic pleural effusions, with congestive heart failure and infection being the other leading causes.[1] The cancers that account for approximately 75% of all malignancy-associated effusions include the following:

Significant use of health care resources is attributable to malignant effusions, with approximately 100,000 cases per year diagnosed in the United States and 43 cases detected per 100,000 hospital admissions.[2]

Pathogenesis

The normal pleural fluid space is occupied with approximately 10 mL of fluid with 2 g/dL protein. A pleural effusion is an accumulation of an abnormal amount of fluid between the visceral and parietal pleura of the chest. Normally, pleural fluid is absorbed by pulmonary venous capillaries (80%–90%), with some of it also absorbed by pleural lymphatics. Malignant effusions are usually exudative rather than transudative. Exudative effusions exhibit any one of the following characteristics:[3]

These exudative malignant effusions are generally caused by:

Paramalignant effusions may result from chemotherapy, radiation therapy, atelectasis, or lymph node involvement.

Assessment

Common symptoms associated with malignant pleural effusions include:

About 20% of patients may experience weight loss and malaise. A chest x-ray is most commonly used for radiographic assessment. About 175 mL of pleural fluid will cause a blunted costophrenic angle discernible on chest radiography. A chest computed tomography scan is more sensitive than a simple chest x-ray and is often used for assessment of loculated effusions because, in some instances, up to 500 mL of loculated fluid can be obscured behind the dome of the diaphragm.[1]

Not all pleural effusions detected in cancer patients are malignant effusions. Cancer patients are prone to developing conditions such as:[1]

Each of these conditions may cause a symptomatic effusion for which clinical management would substantially differ from management of a malignant effusion. For this reason, cytological assessment is important. Pleural fluid cytology requires a minimum sample of 250 mL. The morphology of cells obtained from the pleural space can be difficult to assess because of mesothelial and macrophage abnormalities. The diagnostic sensitivity of pleural fluid cytology is approximately 65%, with a specificity of 97%.[1]

Management of Malignant Pleural Effusions

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.

Incidence and Prevalence

Malignant pericardial effusion occurs in up to 21% of autopsy cases in patients with common malignancies.[4,7] Of patients with lung cancer, 33% have pericardial metastases at autopsy, and one-third of cases of pericardial metastases are caused by lung cancer. Breast cancer causes 25% of pericardial effusions, and about 25% of patients with breast cancer have pericardial effusions. Hematological malignancies (leukemia, Hodgkin disease, non-Hodgkin lymphoma) cause 15% of cases of malignant pericardial effusions.[11]

A retrospective review of 23,592 effusions over a 24-year period revealed 65 malignant effusions (17%) out of 375 pericardial effusions. Lung cancer was the most common cancer found among the malignant pericardial effusions in males, and breast cancer was the most common in females. In 43% of cases, pericardial effusion was the first detected sign of cancer. Of patients diagnosed with malignant pericardial effusions, 86% died within 1 year of diagnosis, with nearly one-third dying within the first month.[8]

In a study of 31 patients with both cancer and pericardial effusions, malignant pericardial effusion accounted for 58% of the effusions, benign idiopathic pericarditis caused 32% of the cases, and radiation pericarditis caused 10% of cases.[11,12]

Pathophysiology

Malignant involvement of the pericardium is the most common reason for development of pericardial effusions, which result from blockage of venous and lymphatic circulation of pericardial fluid. Such blockage may be caused by primary malignancy of the pericardium, such as pericardial mesothelioma, or by tumors arising in the myocardium, including angiosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytosis. Malignancies can also involve the pericardium through direct extension from carcinomas of the lung or esophagus, thymoma, or lymphoma.[9] Lymphatic or hematogenous metastasis to the pericardium occurs most commonly with the following cancers:

Primary tumors of the pleura or pericardium have been termed primary intrathoracic malignant effusions.[13]

Nonmalignant causes of pericardial effusion include:[14–16]

AIDS may also cause pericardial effusion with pericarditis.[17] Radiation therapy or chemotherapy drugs can cause pericarditis without metastatic involvement of the pericardium. Radiation pericarditis is usually associated with radiation doses to the cardiac window exceeding 30 Gy [10] and occurs most frequently in patients who have received mediastinal radiation for Hodgkin lymphoma or breast cancer.[10] Doxorubicin and cyclophosphamide have been associated with the development of acute pericarditis with effusions.[11,12] Other drugs that may cause acute pericarditis include procainamide, hydralazine, isoniazid, methysergide, phenytoin, and anticoagulants.

Pericardial tamponade results from progressive fluid accumulation in the pericardial sac, causing the following:[15]

Hemodynamic compromise occurs when the normal amount of pericardial fluid (approximately 15–50 mL) increases to 200 mL to 1,800 mL.[15,18] When fluid accumulates rapidly, as little as 250 mL of fluid can result in tamponade.[11,19]

Dyspnea occurs in 93% of patients with pericardial effusions.[6] Common symptoms include the following:

Other symptoms of pericardial effusion include:

Signs of effusion include the following:

Pericardial friction rubs and fever are more commonly associated with nonmalignant causes of pericardial effusions than with malignant etiologies.[9]

Signs of pericardial tamponade include:

However, some patients may develop tamponade without this clinical pattern.[4]

Diagnosis

A chest x-ray may show widening of the cardiac silhouette [7] if the amount of pericardial fluid collection exceeds 250 mL.[20] However, a chest x-ray cannot determine the degree of cardiac dysfunction or tamponade. Loculated pericardial effusions may not be apparent on standard posterior/anterior or lateral chest radiographic views.[15]

Transthoracic echocardiography using apical, subxiphoid, and parasternal views can evaluate the presence, quantity, and quality of suspected pericardial effusions, as well as associated pericardial masses and inflammation. Moderate effusions on echocardiography show an echo-free space of 10 mm to 20 mm during diastole in M-mode or 2-dimensional echocardiography, whereas severe effusions have an echo-free space exceeding 20 mm.[21,22] Echocardiography can also determine right and left ventricular function and the possibility of right ventricular or atrial diastolic collapse.[7] Left ventricular collapse caused by large pleural effusions without clinically significant pericardial effusions has been reported.[4,16,23,24] However, transesophageal echocardiography may be useful for loculated effusions resulting from adhesions adjacent to the atria, where the thinness of the atrial wall may not be well visualized on transthoracic echocardiography.[4,16]

Echocardiography in pericardial effusion with tamponade shows right atrial or right ventricular compression, or left atrial compression, decreased left ventricular dimension, and absence of collapse of the inferior vena cava on deep inspiration.[6,25] Echocardiography findings predictive of pericardial tamponade have been reported.[26] Right atrial collapse has a sensitivity of 55% to 60% and a specificity of 50% to 68%. Right ventricular diastolic collapse has a lower sensitivity of 38% to 48% but a higher specificity ranging from 84% to 100%. Because neither finding provides 100% sensitivity and specificity, patients who are clinically symptomatic should have a diagnostic pericardiocentesis, even in the absence of definitive findings on echocardiography.[4,27] One study found right atrial collapse present in only 42% of patients and right ventricular collapse in 62%.[27] Nonetheless, 80% of patients with malignant pericardial effusions had symptomatic relief following pericardiocentesis.

The most definitive test for the diagnosis of cardiac tamponade is equalization of diastolic pressures between all cardiac chambers on right-heart cardiac catheterization.[7] However, this invasive technique is not necessary to diagnose tamponade.

An electrocardiogram (ECG) in patients with pericardial effusions typically shows diminished QRS amplitude in all leads. A classic but uncommonly seen finding in large effusions with pericardial tamponade is variation in the amplitude of the P wave and QRS complex in successive beats on ECG, referred to as electrical alternans. This finding results from movement of the heart within the pericardial sac.[6] Electrocardiography is not sufficiently sensitive to diagnose pericardial effusions.

Pericardial fluid cytology has an accuracy of 80% to 90% in diagnosing malignant pericardial effusion.[6,28] Lymphomas and mesothelioma have higher false-negative detection rates on cytology evaluation.[6,29] Pericardial fluid cytology has a specificity of up to 100%, but sensitivity ranges from 57% to 100% [10][Level of evidence: II] in patients with a known cancer diagnosis and pericardial fluid. Because nonmalignant causes of pericardial effusion can occur in 42% to 62% of patients with cancer and pericardial fluid, a negative cytology examination of pericardial fluid does not help distinguish malignant from nonmalignant causes. The use of more than one cytological preparation (such as concentrating the sample via cytospin, using special markers, or analyzing DNA content) increases the yield over a single preparation. However, multiple samples using the same technique did not significantly increase the diagnostic yield in a retrospective study of 215 patients.[30] In a survey of 80 samples, measurement of DNA index via flow cytometry of pericardial fluid has a sensitivity of 94.8% and a specificity of 100%, compared with routine cytology, which has a sensitivity of 98.5% and a specificity of 92.3%.[31][Level of evidence: II]

Pericardial biopsy may increase the sensitivity of diagnosing pericardial effusions of malignant origin. However, because pericardial effusions usually occur in advanced disease and portend a shorter survival than other sites of metastatic involvement, the relief of symptoms rather than diagnosis should be the overriding factor in determining the extent of the evaluation and the course of treatment. Two studies failed to show a difference in survival in cancer patients with pericardial effusion dependent on the results of fluid cytology.[10,32]

In a study of patients with stage I esophageal cancer who underwent radiation and chemotherapy, risk factors for developing pericardial effusion included advanced age, higher pericardial volume 30 (≥41.6 percentage of cardiac volume receiving more than 30 Gy), high body mass index, and diabetes mellitus.[33]

Treatment

No large controlled, randomized, prospective clinical trials demonstrate the optimal management of malignant pericardial effusions or tamponade. Treatment should therefore be individualized to maximize symptom relief with minimal impact on quality of life. Treatment options include the following:[34][Level of evidence: III]

In choosing a treatment, consider the following:[34][Level of evidence: III]

Large, symptomatic, malignant pericardial effusions are managed by draining the fluid, unless the goals of therapy dictate a less invasive, conservative approach, with concomitant shorter survival balanced against quality-of-life concerns. If treatment is indicated for tamponade, percutaneous subxiphoid pericardiocentesis is the treatment of choice in the acute setting. Echocardiography is recommended for catheter guidance.[6,35] Catheter drainage is recommended for large effusions to prevent rapid reaccumulation of fluid and subsequent tamponade and for the anticipated survival of the patient.

Recurrent pericardial effusion occurs in 21% [36] to 50% [34,35] of patients following pericardiocentesis. Limited case series suggest rates of pericardial fluid reaccumulation at 30 days ranging from 5% to 33% after pericardial drainage followed by intrapericardial treatment with sclerosing agents or phosphorus-colloid, compared with more than 50% for patients treated with pericardial drainage alone.[34,35]

Prolonged catheter drainage can be effective in preventing fluid reaccumulation; however, the mechanism is unclear. One series reported recurrence in 30% of patients at a median time of 39 days. In another series, the reported recurrence rate of the pericardial effusion was 13% by 1 year of follow-up.[37,38]

The prolonged catheter drainage could be left in for several days.[38,39] The catheter should be left in situ until the drainage is minimal (<25–50 mL in a 24-hour period) to none. In one series of 171 patients with malignant pericardial effusion who received echocardiography-guided pericardiocentesis followed by extended catheter drainage, the average time to very little catheter output (<50 mL in 24 hours) was about 3 days.[39] Other treatment options to prevent reaccumulation include intrapericardial sclerosis to obliterate the space within the pericardial sac or pericardiotomy to increase the quantity of fluid drained from the pericardium.

The most effective sclerosing agent for malignant pericardial effusions had been tetracycline, with success rates of up to 80%.[6] However, this agent is no longer available as an intravenous drug in the United States. Alternative sclerosants that have been used include the following:

Most patients may require three or more treatments to achieve adequate sclerosis.[6] Significant pain is reported by 16% of patients undergoing pericardial sclerosis.[6] The side effects of various sclerosing agents (e.g., chest pain and arrhythmias) must be considered. Of patients undergoing pericardial sclerotherapy, 70% to 80% have no fluid reaccumulation within 30 days of the procedure.[35]

A retrospective comparison of pericardiocentesis with sclerotherapy to open surgical drainage in 60 patients showed similar rates of treatment complications, incidence of recurrent effusion, and survival following treatment in both groups.[44] A retrospective review of 59 patients found similar success rates, whether patients were managed with surgical subxiphoid pericardial window or by pericardiocentesis with or without sclerosis.[34] Patients who underwent pericardiocentesis followed by pericardial window had the longest survival, with a median of 6 months. However, selection bias toward patients with better performance status undergoing more aggressive surgical techniques may contribute this reported survival advantage. The surgical procedure group had significantly higher average costs of $4,830 compared with $1,625 for patients managed with pericardiocentesis.[34]

Other studies have reported mortality, recurrence, and survival rates for sclerosis that are similar to or slightly lower than those for subxiphoid window or video-assisted thoracoscopy.[44,45];[46][Level of evidence: II][34][Level of evidence: III] Pericardiocentesis with or without sclerotherapy should be considered instead of more invasive procedures in patients with advanced disease or poor functional status.[47]

Transcutaneous balloon pericardiostomy is another technique that is less invasive than open surgical approaches, which include subxiphoid pericardial windows, thoracotomy with pericardiopleural window formation,[48] and thoracotomy with pericardectomy.

Video pericardioscopy has a diagnostic sensitivity of 97% for detecting malignant effusions.[49] Pericardioscopy also is useful for drainage of loculated effusions.[50][Level of evidence: II] Video-assisted thoracoscopy is preferable to more invasive surgical management and should be considered for patients requiring repeated pericardiocentesis to control symptomatic effusions.[47]

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.

Overview

Superior vena cava syndrome (SVCS) is an array of symptoms caused by the impairment of blood flow through the superior vena cava (SVC) to the right atrium. Symptoms that suggest this syndrome include the following:[1]

In rare instances, patients may complain of hoarseness, chest pain, dysphagia, and hemoptysis.

Physical signs that may be noted on presentation include the following:

Rarely, cyanosis, Horner syndrome, and a paralyzed vocal cord may also be present.[1]

SVCS is usually a sign of locally advanced bronchogenic carcinoma. Survival depends on the status of the patient’s disease. When small cell bronchogenic carcinoma is treated with chemotherapy, the median survival times with or without SVCS are almost identical (42 weeks or 40 weeks, respectively). The 24-month survival rate is 9% in patients without SVCS and 3% in those with the syndrome. When the malignancy is treated with radiation therapy, 46% of patients who have non-small cell lung cancer experience relief of symptoms, compared with 62% of patients who have small cell bronchogenic carcinoma. The 2-year survival rate of 5% is almost the same for both groups.[2]

Most patients with non-Hodgkin lymphoma and SVCS respond to appropriate chemotherapy or to combined-modality regimens.

Etiology and Physiology

Since William Hunter first described SVCS in 1757, the spectrum of underlying conditions associated with it has shifted from tuberculosis and syphilitic aneurysms of the ascending aorta to malignant disorders. Almost 95% of SVCS cases described in published modern series result from cancer. The most common cause is small cell bronchogenic carcinoma, followed by squamous cell carcinoma of the lung, adenocarcinoma of the lung, non-Hodgkin lymphoma, and large cell carcinoma of the lung.[3] Nonmalignant causes of SVCS in cancer patients include thrombosis that is associated with intracaval catheters or pacemaker wires.[4] A rare cause of SVCS is fibrosing mediastinitis, either idiopathic or associated with histoplasmosis.[5] Additional rare causes of SVCS include metastatic germ cell neoplasms, metastatic breast cancer, colon cancer, Kaposi sarcoma, esophageal carcinoma, fibrous mesothelioma, Behçet syndrome, thymoma, substernal thyroid goiter, Hodgkin lymphoma, and sarcoidosis.[6]

Knowledge of the anatomy of the SVC and its relationship to the surrounding lymph nodes is essential to understand development of the syndrome. The SVC is formed by the junction of the left and right brachiocephalic veins in the mid third of the mediastinum. The SVC extends caudally for 6 to 8 cm, coursing anterior to the right mainstem bronchus and terminating in the superior right atrium, and extends anteriorly to the right mainstem bronchus. The SVC is joined posteriorly by the azygos vein as it loops over the right mainstem bronchus and lies posterior to and to the right of the ascending aorta. The mediastinal parietal pleura is lateral to the SVC, creating a confined space, and the SVC is adjacent to the right paratracheal, azygous, right hilar, and subcarinal lymph node groups. The vessel itself is thin-walled, and the blood flowing in it is under low pressure. Thus, when the nodes or ascending aorta enlarge, the SVC is compressed, blood flow slows, and complete occlusion may occur.

The severity of SVCS depends on the speed of onset and location of the obstruction. The more rapid the onset, the more severe the symptoms because the collateral veins do not have time to distend to accommodate an increased blood flow.[7]

One study suggested that the general recruitment of venous collaterals over time may lead to remission of the syndrome, although the SVC remains obstructed.[8]

Assessment and Diagnosis

Once SVCS is recognized, prompt clinical attention is important. A diagnosis should be established before therapy begins because:[3]

In the absence of tracheal obstruction, SVCS is unlikely to be a life-threatening oncologic emergency, and treatment before definitive diagnosis is not justified.

The initial evaluation of the patient should include a chest x-ray to look for mediastinal masses and associated findings, such as pleural effusion, lobar collapse, or cardiomegaly. Computed tomography (CT) scanning of the thorax yields the most useful diagnostic information and can define the anatomy of the involved mediastinal nodes. Venous patency and the presence of thrombi are assessed using contrast and rapid scanning techniques.[9] Depending on local expertise, contrast or nuclear venography, magnetic resonance imaging, and ultrasonography may be valuable in assessing the site and nature of the obstruction.

If bronchogenic carcinoma is suspected, a sputum specimen should be obtained. If the specimen is negative, a biopsy specimen should be taken from the most accessible site that is clinically involved with disease. The biopsy approach depends on the working diagnosis, location of the tumor, physiological status of the patient, and expertise at the facility. It may include:[10]

The biopsy findings will help the clinician plan appropriate treatment.

Treatment Options for SVCS

The treatment of SVCS depends on the following:

Radiation therapy or chemotherapy should be withheld until the etiology of the obstruction is clear. The treatments discussed here focus on SVC obstruction caused by a malignant tumor. Because the treatment of malignant obstruction may depend on tumor histology, a histological diagnosis should be made before starting treatment. Unless there is airway obstruction or cerebral edema, treatment may be delayed until after an assessment, with no negative effect on outcome.[1,11–15]

Psychosocial Considerations

Patients and family members are often frightened and anxious about the symptoms of SVCS, particularly swelling, dysphagia, coughing, and hoarseness. Patients and family members need information about the cause of the symptoms and short-term measures for palliation, especially during the diagnostic period. When aggressive treatment is declined because of the terminal nature of the underlying disease, symptom management can be offered to patients and family members.

Because most adult patients who develop SVCS have lung cancer, the treatment and psychological support approaches for SVCS should take into account the patient’s prognosis and mental condition, goals of care, and other symptoms caused by the malignancy.[25]

Pediatric Considerations

As described in this summary, SVCS refers to the symptoms associated with the compression or obstruction of the SVC; the compression of the trachea is termed superior mediastinal syndrome (SMS). Because SMS and the resulting respiratory compromise frequently occur in children with SVCS, the two syndromes have become almost synonymous in pediatric practice.[26,27] In adults, the trachea and the right mainstem bronchus are relatively rigid structures compared with the vena cava, but in children, these structures are more susceptible to compression. In addition, the relatively smaller intraluminal diameters of a child’s trachea and bronchus can tolerate little edema before respiratory symptoms occur. Because of this accompanying respiratory component, SVCS in children differs from the adult syndrome and is a serious medical emergency.

The most common symptoms of SVCS in children are similar to those in adults and include the following:[26]

Less common but more serious symptoms include the following:

SVCS is rare in children, appearing at presentation in 12% of pediatric patients with malignant mediastinal tumors.[28,29] The etiology, diagnosis, and treatment of SVCS in children differ from those in adults. The most frequent cause of SVCS in adults is bronchogenic carcinoma;[3] in children, it is non-Hodgkin lymphoma. As in adults, a frequent nonmalignant cause is thrombosis from catheterization for venous access.[26]

A physical examination, chest radiograph, and medical history are usually sufficient to diagnose SVCS. If lymphomas or other malignant diseases are suspected, a tissue sample may aid diagnosis. However, the procedure to obtain the specimen may involve significant risk and may not be clinically feasible. Children with SVCS have a poor tolerance for the necessary general anesthesia because the accompanying cardiovascular and pulmonary changes aggravate the SVCS, often making intubation impossible. Also, extubation may be difficult or impossible, requiring prolonged airway provision (intubation). A CT scan of the chest to determine tracheal size, upright and supine echocardiography, and a flow volume loop may help evaluate anesthetic risk. Because anesthesia use is a serious risk, the diagnosis should be made with the least invasive means possible.[30] Published reports suggest a stepwise approach.[26]

When a malignant mass causes SVCS, the situation may be a medical emergency with no opportunity to establish a tissue diagnosis. In these cases, the most appropriate course may be to initiate empiric therapy before biopsy. The traditional empiric therapy is radiation therapy, with the daily dose governed by the presumed radiosensitivity of the tumor. After radiation therapy, respiratory deterioration from the apparent tracheal swelling may occur. This swelling occurs because narrow lumens in children cannot accommodate edema and because of the greater degree of edema at onset, the result of rapid tumor growth in children. In these situations, a course of prednisone at 10 mg/m2 of body surface area 4 times per day may be necessary.[26]

In addition to radiation, empiric therapy for SVCS has included chemotherapeutic agents incorporating steroids, cyclophosphamide, or both in combination with an anthracycline and vincristine.[26] If the tumor fails to respond, it may be a benign lesion.

If surgery becomes necessary, it should be performed with the patient in the semi-Fowler’s position, allowing the surgeon the ability to rapidly change the patient’s position to lateral or prone. Cardiopulmonary bypass facilities and a rigid bronchoscope should be available in a standby capacity.[30]

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the pathophysiology and treatment of cardiopulmonary syndromes, including dyspnea, malignant pleural effusion, malignant pericardial effusion, and superior vena cava syndrome. 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 Supportive and Palliative Care 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.

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 Supportive and Palliative Care 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® Supportive and Palliative Care Editorial Board. PDQ Cardiopulmonary Syndromes. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /side-effects/cardiopulmonary-hp-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389275]

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

The information in these summaries 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.

Many conditions can cause dyspnea.

Dyspnea is the feeling that you can’t catch your breath or you can’t breathe in enough air. It also may be called shortness of breath, breathlessness, or air hunger. In people with cancer, dyspnea can be caused by:

A physical exam and health history are important in finding out the cause of dyspnea.

Diagnostic tests and procedures include:

There are different ways to treat the causes of dyspnea in people with cancer.

Treatment may include:

Treatment of dyspnea will depend on what is causing it.

The treatment of dyspnea depends on its cause, as follows:

Treatment may be to control the signs and symptoms of dyspnea.

Treatment to control the signs and symptoms of dyspnea may include:

Chronic coughing may cause much physical distress.

Chronic cough may cause pain, trouble sleeping, dyspnea, or fatigue. The causes of chronic coughing are almost the same as the causes of dyspnea. Learn more in the Dyspnea section.

It may be possible to treat the cause of chronic coughing.

Treatments may include:

Medicines may be used to control chronic coughing.

Medicines may include:

Pleural effusion is extra fluid in the pleural cavity.

The pleural cavity is the space between the pleura (thin layer of tissue) that covers the outer surface of each lung and lines the inner wall of the chest cavity. Pleural tissue usually makes a small amount of fluid that helps the lungs move smoothly in the chest while a person is breathing. A pleural effusion is extra fluid in the pleural cavity. The fluid presses on the lungs and makes it hard to breathe.

Pleural effusion may be caused by cancer, cancer treatment, or other conditions.

A pleural effusion may be malignant (caused by cancer) or nonmalignant (caused by a condition that is not cancer). Malignant pleural effusion is a common problem for people with certain cancers. Lung cancer, breast cancer, lymphoma, and leukemia cause most malignant effusions.

Pleural effusion also may be caused by radiation therapy, chemotherapy, a collapsed lung, or cancer that has spread to lymph nodes. Some people with cancer have conditions such as congestive heart failure, pneumonia, blood clot in the lung, or poor nutrition that may lead to a pleural effusion.

Signs and symptoms of pleural effusion include dyspnea (shortness of breath) and cough.

These and other signs and symptoms may be caused by a pleural effusion. Talk to your doctor if you have:

Finding out the cause of pleural effusion will help plan the treatment.

Treatment for a malignant pleural effusion is different from treatment for a nonmalignant effusion, so the right diagnosis is important. Diagnostic tests used to find the cause of the pleural effusion include:

The type of cancer, previous treatment for cancer, and your choices also are important in planning treatment.

Treatment may be to control signs and symptoms of pleural effusion and improve quality of life.

A malignant pleural effusion often occurs in cancer that is advanced, cannot be removed by surgery, or continues to grow or spread during treatment. It is also common during the last few weeks of life. The goal of treatment is usually palliative, to relieve signs and symptoms and improve quality of life.

Treatment of the signs and symptoms of malignant pleural effusion includes:

Pericardial effusion is extra fluid inside the sac around the heart.

Pericardial effusion is extra fluid inside the sac around the heart. The extra fluid causes pressure on the heart, which stops it from pumping blood normally. If fluid builds up, a condition called cardiac tamponade may occur. In cardiac tamponade, the heart cannot pump enough blood to the rest of the body. This is life-threatening and must be treated right away.

Pericardial effusion may be caused by cancer or other conditions.

A pericardial effusion may be malignant (caused by cancer) or nonmalignant (caused by a condition that is not cancer). Malignant pericardial effusion is common in people with lung cancer, breast cancer, melanoma, lymphoma, and leukemia. Pericarditis (swelling of tissues around the heart), a heart attack, hypothyroidism, or systemic lupus erythematosus are examples of nonmalignant causes of pericardial effusion. Radiation therapy or chemotherapy may cause pericarditis, leading to pericardial effusion.

Signs and symptoms of pericardial effusion include dyspnea (shortness of breath) and cough.

At first, a pericardial effusion may not cause any signs or symptoms. These and other signs and symptoms may be caused by a pericardial effusion or cardiac tamponade. Check with your doctor if you have:

Pericardial effusion usually occurs in advanced cancer.

Pericardial effusion usually occurs in advanced cancer or in the last few weeks of life. During these times, it may be more important to relieve the symptoms than to diagnose the condition. However, in some cases, the following tests and procedures may be used to diagnose pericardial effusion:

Treatment may be to control the symptoms of pericardial effusion and improve quality of life.

The goal of treatment is usually palliative, to relieve symptoms and improve quality of life. If a malignant pericardial effusion is severe, it is usually controlled by draining the fluid.

Treatment options include:

Superior vena cava syndrome (SVCS) is a group of signs and symptoms that occur when the superior vena cava is partly blocked.

The superior vena cava is a major vein that leads to the heart. The heart is divided into four parts. The right and left atrium make up the top parts of the heart and the right and left ventricle make up the bottom parts of the heart. The right atrium of the heart receives blood from two major veins:

Different conditions can slow the flow of blood through the superior vena cava. These include a tumor in the chest, nearby lymph nodes that are swollen (from cancer), or a blood clot in the superior vena cava. The vein may become completely blocked. Sometimes, smaller veins in the area become larger and take over for the superior vena cava if it is blocked, but this takes time. Superior vena cava syndrome (SVCS) is the group of signs and symptoms that occur when this vein is partly blocked.

SVCS is usually caused by cancer.

SVCS is usually caused by cancer. In adults, SVCS is most common in the following types of cancer:

Less common causes of SVCS include:

Common signs and symptoms of SVCS include breathing problems and coughing.

The signs and symptoms of SVCS are more severe if the vein becomes blocked quickly. This is because the other veins in the area do not have time to widen and take over the blood flow that cannot pass through the superior vena cava.

The most common signs are:

Less common signs and symptoms include:

Tests are done to find and diagnose the blockage.

The following tests may be done to diagnose SVCS and find the blockage:

It is important to find out the cause of SVCS before starting treatment. The type of cancer can affect the type of treatment needed. Unless the airway is blocked or the brain is swelling, waiting to start treatment while a diagnosis is made usually causes no problem in adults. If doctors think lung cancer is causing the problem, a sputum sample may be taken and a biopsy may be done.

Treatment for SVCS caused by cancer depends on the cause, signs and symptoms, and prognosis.

Treatment for SCVS caused by cancer depends on:

Treatments for SVCS include watchful waiting, chemotherapy, radiation therapy, thrombolysis, stent placement, and surgery.

Palliative care may be given to relieve signs and symptoms of SVCS.

The signs and symptoms of superior vena cava syndrome can be upsetting. It is important that you and your family ask questions about superior vena cava syndrome and how to treat it. This can help relieve anxiety about signs and symptoms such as swelling, trouble swallowing, coughing, and hoarseness.

If you have advanced cancer you may decide not to have any more treatment. Palliative care can help keep you comfortable by relieving signs and symptoms to improve your quality of life.

Superior vena cava syndrome (SVCS) in a child is a serious medical emergency because the child’s windpipe can become blocked.

Superior vena cava syndrome (SVCS) is rare in children, but when it occurs, it can be life-threatening. In adults, the windpipe is stiff, but in children, it is softer and can more easily be squeezed shut or blocked. A child’s windpipe is narrow, so any amount of swelling can cause breathing problems. Squeezing of the trachea is called superior mediastinal syndrome (SMS). Because SVCS and SMS usually happen together in children, the two syndromes are considered to be the same.

The most common symptoms of SVCS in children are a lot like those in adults.

Common signs and symptoms include:

There are other less common but more serious signs and symptoms:

The most common cause of SVCS in children is non-Hodgkin lymphoma.

SVCS in children is rare. The most common cause is non-Hodgkin lymphoma. As in adults, SVCS may also be caused by a blood clot that forms during use of an intravenous catheter (flexible tube used to put fluids into or take blood out of a vein) in the superior vena cava.

SVCS in children may be diagnosed and treated before a diagnosis of cancer is made.

A physical exam, chest x-ray, and medical history are usually all that are needed to diagnose superior vena cava syndrome in children. Even if doctors think cancer is causing SVCS, a biopsy may not be done. This is because the lungs and heart of a child with SVCS may not be able to handle the anesthesia needed. Other imaging tests may be done to help find out if anesthesia can be safely used. In most cases, treatment for SVCS will begin before a diagnosis of cancer is made.

Treatment for SVCS in children may include radiation therapy, drugs, or surgery.

There are several ways to treat SVCS in children.

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 pathophysiology and treatment of cardiopulmonary syndromes, including dyspnea, malignant pleural effusion, malignant pericardial effusion, and superior vena cava syndrome. 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 Supportive and Palliative Care 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® Supportive and Palliative Care Editorial Board. PDQ Cardiopulmonary Syndromes. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /side-effects/cardiopulmonary-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389457]

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.