Version May 2024
INTRODUCTION — Cancer commonly involves the pericardium, with clinical presentations ranging from acute syndromes to indolent conditions.
The clinical presentation and diagnosis of pericardial disease associated with cancer is reviewed here. The management of pericardial disease associated with cancer is discussed separately. (See "Pericardial disease associated with cancer: Management".)
The etiology of pericardial disease and the diagnosis of pericardial effusion, cardiac tamponade, and constrictive pericarditis in the general population are also presented separately.
●(See "Etiology of pericardial disease".)
●(See "Pericardial effusion: Approach to diagnosis".)
●(See "Cardiac tamponade".)
●(See "Constrictive pericarditis: Diagnostic evaluation".)
EPIDEMIOLOGY
●Pericardial disease in patients with cancer — Pericardial disease occurs in approximately 10 to 20 percent of patients with cancer [1,2]. The frequency of pericardial disease caused by cancer and other conditions varies depending upon the type of cancer and associated complications. (See 'Etiology' below.)
In autopsy series of patients with cancer, cardiac or pericardial involvement has been reported in approximately 10 percent of cases [1,3-5]. Of these cases, the epicardium/pericardium was the most common cardiac site of involvement (76 percent), followed by the myocardium (38 percent) and endocardium (16 percent) [2,3,6].
●Cancer in patients with pericardial disease — Acute pericarditis and/or pericardial effusion is associated with an elevated risk of subsequent cancer diagnosis, particularly during the first three months after identification of pericardial disease. In a population-based matched cohort study from the United Kingdom, the incidence of cancer detection was higher among 2221 patients diagnosed with a pericardial effusion than among 11,538 patients diagnosed with acute pericarditis without pericardial effusion (hazard ratio [HR] 3.59, 95% CI 3.18-4.06 versus HR 2.04, 95% CI 1.69-2.46) [7]. Patients with pericardial effusion or acute pericarditis and need for hospitalization, older age, obesity, and/or current smoking had the highest absolute excess risks of cancer identified 3 to 12 months after diagnosis of pericardial disease.
Among patients who present with pericardial disease, the likelihood of identifying a previously undiagnosed cancer is higher among patients who present with a moderate or large pericardial effusion (table 1), cardiac tamponade [8,9], or effusive-constrictive pericarditis than in those who present with acute pericarditis, a small pericardial effusion, or constrictive pericarditis. These clinical syndromes are discussed below. (See 'Clinical presentation' below.)
ETIOLOGY — Clinical conditions that cause pericardial disease in patients with cancer include [8,10,11]:
●Malignancy (see 'Malignancy' below)
●Cancer therapy (eg, radiation therapy [RT], antineoplastic agents) (see 'Cancer therapy' below)
●Infection (see 'Infection' below)
●Idiopathic pericarditis (see "Etiology of pericardial disease", section on 'Idiopathic')
Further details on other etiologies of pericardial disease in the general population are discussed separately. (See "Etiology of pericardial disease".)
Malignancy — Malignancy (local invasion, metastases, or mediastinal lymphatic obstruction) is a common cause of pericardial disease in patients with cancer. In observational studies in patients with cancer and pericardial effusion undergoing pericardiocentesis, the frequency of a malignant effusion is approximately 40 to 60 percent [1,12-15].
Metastatic disease — Malignant tumors can involve the pericardium either by direct local invasion or metastatic spread via the lymphatics or bloodstream. Metastatic disease of the pericardium most commonly presents with pericardial effusion (with or without cardiac tamponade). Metastatic disease less commonly presents as constrictive pericarditis, which is a rare initial manifestation of cancer [16].
Lung cancer is the most common malignancy to involve the pericardium. In particular, patients with non-small cell lung cancer that harbors an anaplastic lymphoma kinase (ALK) rearrangement are particularly likely to present with a pericardial effusion or other pericardial involvement [17]. (See "Anaplastic lymphoma kinase (ALK) fusion oncogene positive non-small cell lung cancer", section on 'Clinicopathologic features'.)
Other common cancers that can cause pericardial disease include breast and esophageal cancer, melanoma, lymphoma, and leukemia [3,4,12,13,18-20]. Although acquired immunodeficiency syndrome (AIDS)-related Kaposi sarcoma (KS) was previously an important cause of malignant pericardial disease [21,22], the incidence of KS has declined dramatically with widespread use of potent combination antiretroviral therapy. (See "AIDS-related Kaposi sarcoma: Clinical manifestations and diagnosis", section on 'Epidemiology and risk factors' and "Cardiac and vascular disease in patients with HIV", section on 'Pericardial disease'.)
Primary pericardial tumors — Primary malignancies arising in the pericardium are extremely rare [2]. These include mesotheliomas, teratomas, and paragangliomas. The differential diagnosis for these conditions includes benign lesions such as pericardial cysts and lipomas. These conditions are discussed separately. (See "Cardiac tumors".)
Cancer therapy — Cancer treatment, such as RT or antineoplastic agents, is frequently associated with pericardial disease in patients with cancer. In one observational study of patients undergoing pericardiocentesis for pericardial effusion, cancer therapy was identified as a likely etiology in almost 60 percent of patients with pericardial disease and cancer [15].
Radiation — RT to the chest is a cause of acute pericarditis, with or without pericardial effusion. Patients may present days to weeks after initiating RT. RT less commonly causes constrictive pericarditis, which may manifest months to years after therapy is completed [23,24]. Radiation-induced pericardial disease is most commonly caused by RT for Hodgkin lymphoma, breast, esophageal, or lung cancer. (See 'Clinical presentation' below.)
The risk of radiation-induced pericarditis has declined with contemporary RT techniques [24-27]. However, high rates of pericardial effusion have been reported in observational series of patients treated with RT for lung or esophageal cancer, although many of the pericardial effusions were asymptomatic [28,29]. A systematic review found that symptomatic pericardial effusion typically occurs with cardiac RT doses greater than 40 Gy [30]. Another study of patients with esophageal cancer found that pericarditis occurred only in patients who had received a dose of 3.5 Gy daily fraction; thus, a daily limit of 2 Gy daily fraction to the heart was suggested [31]. (See "Cardiotoxicity of radiation therapy for Hodgkin lymphoma and pediatric malignancies", section on 'Pericarditis' and "Cardiotoxicity of radiation therapy for breast cancer and other malignancies".)
Cancer-directed therapy — Antineoplastic agents that can cause pericarditis or pericardial effusion include cytotoxic chemotherapy (eg, fludarabine, cytarabine, bleomycin, anthracyclines, docetaxel, and cyclophosphamide), and other targeted agents (eg, all-trans retinoic acid, arsenic trioxide, dasatinib) [11,32-34]. Immune checkpoint inhibitors are a rare cause of acute pericarditis and pericardial effusion, which is often complicated by cardiac tamponade [35-37]. (See "Toxicities associated with immune checkpoint inhibitors", section on 'Cardiovascular toxicity'.)
Infection — Infectious etiologies (viral, bacterial, fungal) are relatively uncommon causes of pericardial diseases in patients with cancer (approximately 2 percent) [15] However, infectious pericardial disease can be associated with high mortality in patients who are immunocompromised, especially when the etiology is fungal infection [38]. (See "Etiology of pericardial disease" and "Purulent pericarditis" and "Tuberculous pericarditis".)
CLINICAL PRESENTATION — In patients with cancer, pericardial disease can present as acute pericarditis, pericardial effusion with or without cardiac tamponade, constrictive pericarditis, or effusive-constrictive pericarditis [7,39-47].
Acute pericarditis — Acute pericarditis can present with or without a pericardial effusion. It is unusual for malignant involvement of the pericardium to present with acute pericarditis without an associated pericardial effusion or pericardial mass.
Among patients presenting with acute pericarditis or a small pericardial effusion without known cancer, the likelihood of identifying an undiagnosed cancer ranges between approximately 3 and 7 percent [7,39-43].
Acute pericarditis can present with a variety of nonspecific signs and symptoms, depending on the underlying etiology. Common clinical manifestations include pleuritic chest pain, fever, pericardial friction rub, and diffuse ST elevation and PR depression on the electrocardiogram (ECG) (waveform 1).
Further details on the diagnosis and treatment of acute pericarditis are discussed separately. (See "Acute pericarditis: Clinical presentation and diagnosis" and "Acute pericarditis: Treatment and prognosis".)
Pericardial effusion — Patients with cancer commonly have pericardial effusions. Among patients with cancer and a pericardial effusion requiring drainage, approximately 50 percent will have a malignant effusion, although this percentage varies depending upon the type of cancer and exposures to other causes of pericardial effusion.
Among patients without a known cancer, the likelihood of a malignant pericardial effusion is higher in the presence of moderate or large pericardial effusion than with a small pericardial effusion. In a series of 57 patients with a new large effusion, a specific diagnosis was established by pericardial biopsy and/or fluid cytology in 93 percent; 13 patients (23 percent) were newly diagnosed with cancer [47]. (See 'Epidemiology' above.)
Pericardial effusion may occur with or without associated pericarditis. The presence of pericardial effusion is generally confirmed by echocardiography, although additional cardiac imaging is required in selected cases. (See "Pericardial effusion: Approach to diagnosis" and 'Further cardiac imaging' below.)
With cardiac tamponade — Some patients with malignant pericardial effusion develop cardiac tamponade. In cardiac tamponade, the pressure from the accumulating pericardial effusion approaches and then equalizes with intracardiac pressures, leading to impaired filling of one or both ventricles and decreased cardiac output. Cardiac tamponade may be caused by a circumferential or loculated pericardial effusion; the latter may be missed in standard parasternal and apical echocardiographic views. The clinical features, diagnosis, and management of cardiac tamponade are discussed separately. (See "Cardiac tamponade".)
Malignant pericardial effusion is a common cause of cardiac tamponade. This was illustrated in an observational study of 173 patients undergoing pericardiocentesis for hemodynamically significant pericardial effusion, among whom most (84 percent) were diagnosed with tamponade [9]. The most common etiology was malignancy, which was diagnosed in 58 patients (33 percent). Among those 58 patients with malignant pericardial effusion, 45 patients had known malignant disease and 13 patients had newly diagnosed cancer. Tamponade with hemorrhagic pericardial effusion was more common in patients with malignant effusions than in those with other causes of pericardial disease.
The clinical features of cardiac tamponade vary depending upon whether the onset of fluid accumulation is acute or subacute. In the setting of cancer, cardiac tamponade is most commonly subacute.
●Acute cardiac tamponade – Acute cardiac tamponade is generally rapid in onset, may be associated with chest pain and dyspnea, and is life-threatening if not promptly treated. With rapid introduction of even a small amount of fluid into the pericardial space, the parietal pericardium cannot acutely stretch to accommodate the increase in volume. This results in an acute rise in pericardial pressure, which is transmitted to all cardiac chambers and limits diastolic filling. Common clinical findings include tachycardia, hypotension, narrow pulse pressure, jugular venous distention, and pulsus paradoxus (an accentuated inspiratory decrease in systolic blood pressure).
●Subacute cardiac tamponade – Subacute cardiac tamponade is a less dramatic process. Patients may be asymptomatic or complain of one or more of the following: fatigue, dyspnea, chest discomfort or fullness, and edema. Hypotension may or may not be present. Low pressure (occult) cardiac tamponade is a subset of subacute cardiac tamponade in which the patient is severely hypovolemic.
A minority of patients with cardiac tamponade have effusive-constrictive pericarditis, as discussed below. (See 'Effusive-constrictive pericarditis' below.)
Without cardiac tamponade — Most patients without a hemodynamically significant pericardial effusion (ie, without cardiac tamponade) will have no symptoms specific to the effusion, but they may have symptoms related to the underlying cause (eg, fever in the setting of pericarditis). The pericardial effusion may be acute (recent onset), recurrent, or chronic. (See "Pericardial effusion: Approach to diagnosis".)
Constrictive pericarditis — Constrictive pericarditis, sometimes referred to as pericardial constriction, generally results from scarring and fibrosis of the visceral and/or parietal pericardium, which progressively restricts diastolic filling of the ventricles. Patients with constrictive pericarditis typically present with one or both of the following constellations of symptoms: symptoms related to fluid overload, ranging from peripheral edema to anasarca; and/or symptoms related to diminished cardiac output in response to exertion, such as fatigability and dyspnea on exertion. (See "Constrictive pericarditis: Clinical features and causes" and "Constrictive pericarditis: Diagnostic evaluation".)
Patients presenting with constrictive pericarditis have a low likelihood of harboring an undiagnosed malignancy. In observational studies of patients with constrictive pericarditis, malignancy was identified in fewer than 3 percent [44-46]. Most cases of constrictive pericarditis in resource abundant countries are idiopathic, related to prior cardiac surgery, or caused by radiation; malignancy is an uncommon cause. Tuberculosis is a common cause of constrictive pericarditis in resource-limited countries.
However, a significant percentage of patients with constrictive pericarditis have been previously treated with radiation therapy (RT) for malignancy; in observational series of patients with constrictive pericarditis, RT (most commonly used to treat Hodgkin lymphoma or breast cancer) was the suspected cause in 9 to 31 percent of patients [44-46]. (See "Constrictive pericarditis: Diagnostic evaluation".)
Effusive-constrictive pericarditis — In this condition, there is both a hemodynamically significant pericardial effusion and pericardial constriction. Drainage of pericardial fluid may unmask findings typical of constrictive physiology. Most cases of effusive-constrictive pericarditis are idiopathic; radiation and cancer are less common causes [48,49]. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Effusive-constrictive pericarditis'.)
DIAGNOSTIC EVALUATION FOR PERICARDIAL DISEASE
General approach — For patients with a known or suspected cancer and suspected pericardial disease, our approach is detailed below. (See 'Initial evaluation' below.)
For patients without a known cancer, pericardial disease may be the first manifestation of malignancy. Patients who should undergo a detailed evaluation for occult cancer include those with persistent or recurrent pericarditis that is unresponsive to antiinflammatory therapy, those in whom cancer is suspected (eg, unexplained weight loss or a heavy smoking history), and those who present with a new large pericardial effusion or cardiac tamponade. (See "Pericardial effusion: Approach to diagnosis" and "Acute pericarditis: Clinical presentation and diagnosis" and "Constrictive pericarditis: Diagnostic evaluation" and "Overview of the classification and management of cancers of unknown primary site".)
Initial evaluation — Patients with suspected malignant pericardial disease are initially evaluated by history, physical examination, ECG, chest radiograph, and transthoracic echocardiogram.
Laboratory tests
●Acute pericarditis – In patients with suspected acute pericarditis, certain markers of inflammation (white blood cell count, erythrocyte sedimentation rate, and serum C-reactive protein) are often elevated, whether or not malignant pericardial disease is present. Further details on initial laboratory testing for acute pericarditis are discussed separately. (See "Acute pericarditis: Clinical presentation and diagnosis", section on 'Diagnostic evaluation'.)
●Pericardial effusion – The approach to initial laboratory testing for suspected pericardial effusion is discussed separately. (See "Pericardial effusion: Approach to diagnosis", section on 'Laboratory tests'.)
●Constrictive pericarditis – The approach to initial laboratory testing for suspected constrictive pericarditis is discussed separately. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Initial tests'.)
Electrocardiogram — An ECG may show changes suggestive of pericardial disease, regardless of the etiology of disease. The ECG may also show atrial fibrillation, which is common in patients with pericardial disease, particularly those with constrictive pericarditis.
●Acute pericarditis – In patients with acute pericarditis, characteristic ECG findings include diffuse ST elevation (typically concave up) with reciprocal ST depression in leads aVR and V1 (waveform 1). There is also frequently an atrial current of injury, reflected by elevation of the PR segment in lead aVR and depression of the PR segment in other limb leads and in the left chest leads, primarily V5 and V6. Thus, the PR and ST segments typically change in opposite directions. (See "Acute pericarditis: Clinical presentation and diagnosis", section on 'Electrocardiogram'.)
●Pericardial effusion – In patients with a pericardial effusion, especially if cardiac tamponade is present, QRS voltage may be decreased and, occasionally, electrical alternans is seen (waveform 2). This abnormality is characterized by a cyclic beat-to-beat shift in the QRS axis associated with mechanical swinging of the heart to-and-fro, usually in a large pericardial effusion. (See "Pericardial effusion: Approach to diagnosis", section on 'ECG findings'.)
●Constrictive pericarditis – There are no diagnostic ECG findings for constrictive pericarditis or effusive-constrictive pericarditis. Further details on ECG findings for this condition are discussed separately. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Electrocardiogram'.)
Chest radiograph — A chest radiograph is a standard test in the evaluation of suspected pericardial disease, although it has limited sensitivity and specificity for detection of pericardial disease and causes of pericardial disease such as cancer.
●Acute pericarditis – A chest radiograph is typically normal with acute pericarditis with no or small pericardial effusion. (See "Acute pericarditis: Clinical presentation and diagnosis", section on 'Chest radiograph'.)
●Pericardial effusion – A large pericardial effusion may manifest as an enlarged cardiac silhouette on chest radiograph. In patients with subacute cardiac tamponade, an enlarged cardiac silhouette with clear lung fields may be seen. However, an enlarged cardiac silhouette is not specific for pericardial effusion as it may also be caused by cardiomegaly. (See "Pericardial effusion: Approach to diagnosis", section on 'Chest radiograph'.)
A chest radiograph is also useful for detecting a concomitant pleural effusion. In a surgical series of 47 patients treated for symptomatic pericardial effusion, 41 (87 percent) had a concomitant pleural effusion and 20 (42 percent) of these were cytologically positive for cancer (image 1) [50].
●Constrictive pericarditis – Pericardial calcifications in the presence of typical symptoms are strongly suggestive of constrictive pericarditis, although most patients with constrictive pericarditis do not have pericardial calcification on chest radiograph. Pericardial calcification is identified in a minority of patients with constrictive pericarditis caused by radiation therapy (RT) [51]. Although pericardial calcification is not generally associated with malignant pericardial disease, its presence does not exclude cancer [52]. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Chest radiograph'.)
Echocardiogram — All patients with suspected pericardial disease should have a transthoracic echocardiogram. Echocardiography is the primary imaging tool used to establish the presence and quantity of a pericardial effusion and to evaluate its hemodynamic impact, particularly the presence of cardiac tamponade or constrictive physiology. For patients not requiring immediate therapeutic intervention, echocardiography is also the primary means to monitor changes in hemodynamic status or effusion size. (See "Pericardial effusion: Approach to diagnosis", section on 'Cardiac imaging' and "Constrictive pericarditis: Diagnostic evaluation" and "Cardiac tamponade".)
Additional testing — The need for additional testing is determined by the results of the initial evaluation. Additional testing may include further cardiac imaging, cardiac catheterization to assess hemodynamics, and evaluation of pericardial fluid and pericardial tissue. (See 'Cytology' below and 'Flow cytometry' below and 'Indications for obtaining a pericardial specimen' below.)
●Cardiac tamponade – If there are symptoms and signs of cardiac tamponade, echocardiography is generally diagnostic. However, further imaging may be required if the transthoracic echocardiogram is technically suboptimal or negative but a loculated pericardial effusion or pericardial hematoma is suspected. (See 'Further cardiac imaging' below.)
Patients with cardiac tamponade require urgent pericardial drainage or evacuation with analysis of pericardial fluid. (See 'Pericardial effusion' below and "Pericardial effusion: Approach to diagnosis".)
●Pericardial effusion without tamponade – If a pericardial effusion is identified, and there is no evidence of cardiac tamponade, pericardial fluid sampling or a pericardial biopsy may be helpful to document potential neoplastic disease involving the pericardium, particularly if there is no preexisting diagnosis of cancer. (See 'Cytology' below.)
●Constrictive pericarditis – If there are symptoms and signs of constrictive pericarditis, cardiovascular magnetic resonance imaging (CMR), cardiac computed tomography (CT), and/or right heart catheterization may be needed to confirm the diagnosis.
Further cardiac imaging — Cardiac CT or CMR is obtained if initial testing, including echocardiography, is nondiagnostic, but pericardial disease is still suspected [53]. Indications include:
●Loculated or hemorrhagic pericardial effusion – While a transthoracic echocardiogram is generally sufficient to identify most pericardial effusions, CMR or cardiac CT is indicated if the echocardiogram is technically suboptimal or negative but a loculated and/or hemorrhagic pericardial effusion is suspected [54]. Both cardiac CT and CMR are helpful in identifying pericardial effusions and chamber collapse seen with cardiac tamponade (image 2). (See "Pericardial effusion: Approach to diagnosis", section on 'Cardiac imaging'.)
These modalities also have the advantage of providing information about the remainder of the chest as well (eg, detecting the presence of a primary tumor or metastatic foci within lung parenchyma). In some cases (particularly in the setting of trauma), a hematoma may be mistaken for a neoplasm. (See "Clinical utility of cardiovascular magnetic resonance imaging", section on 'Pericardial disease'.)
●Constrictive pericarditis – For patients with suspected constrictive pericarditis with nondiagnostic echocardiography, CMR or cardiac CT may be helpful as these modalities provide more accurate measures of pericardial thickness (image 3 and image 4) and may also identify other features of pericardial constriction (algorithm 1). Increased pericardial thickness (>2 mm) is suggestive of but not diagnostic for constrictive pericarditis. However, absence of pericardial thickening does not exclude constrictive pericarditis. (See "Constrictive pericarditis: Diagnostic evaluation" and "Constrictive pericarditis: Diagnostic evaluation", section on 'Cardiac CT or CMR'.)
Diffuse, late pericardial gadolinium enhancement on CMR is a sign of pericarditis with active inflammation; less commonly, it is a sign of diffuse neoplastic involvement [55].
●Pericardial mass – When a pericardial mass is suspected, cardiac CT (image 5) or CMR provide greater field of view and anatomic information than echocardiography, including identification of nodal and other extracardiac involvement. Additionally, cross-sectional imaging is frequently a part of the staging/restaging process of the cancer. Both abdominal and chest CT can provide information about the pericardial space (image 6).
Patients with a confirmed diagnosis of cancer may also require further systemic imaging for staging or restaging evaluation.
Cardiac catheterization — Cardiac catheterization may be obtained if testing, including echocardiography and other cardiac imaging, is inconclusive or equivocal for identifying cardiac tamponade or constrictive physiology (constrictive pericarditis versus restrictive cardiomyopathy). Cardiac catheterization is also used to monitor intracardiac pressures during pericardiocentesis. (See "Pericardial effusion: Approach to diagnosis".)
Constrictive pericarditis is often difficult to distinguish from restrictive cardiomyopathy based upon clinical and echocardiographic criteria alone. Patients with prior chest radiation may develop constrictive pericarditis and/or restrictive cardiomyopathy. Cardiac catheterization can be used to distinguish between these two conditions using hemodynamic assessment with simultaneous right and left heart pressure measurement, particularly examination of respirophasic changes in pressures, to characterize the impact of pericardial disease upon cardiac filling and performance [56]. Of note, RT can cause constrictive pericarditis and/or restrictive cardiomyopathy.
Further details on the use of cardiac catheterization in this setting are discussed separately. (See "Constrictive pericarditis: Diagnostic evaluation" and "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Cardiac catheterization'.)
DIAGNOSIS OF MALIGNANT PERICARDIAL DISEASE — The diagnosis of malignant pericardial disease is typically confirmed on pathology, using either cytology (with or without flow cytometry) of the pericardial fluid or pericardial tissue biopsy.
Pericardial effusion
Cytology — In patients with pericardial effusion of uncertain etiology who have undergone pericardial fluid sampling, cytologic examination is an initial step in identifying a malignant cause. Hemorrhagic effusions are more likely to be malignant than nonhemorrhagic effusions. Therefore, cytologic evaluation is especially important if the effusion is hemorrhagic and there is no history of antecedent trauma [57]. (See "Pericardial effusion: Approach to diagnosis".)
The sensitivity of cytology for the diagnosis of a malignant effusion is between 67 and 92 percent and is lowest for mesothelioma and lymphoma [12,14,58-60]. Immunohistochemical staining may help distinguish malignant cells from atypical mesothelial cells as well as identify the likely tissue of origin if malignant cells are present [61-64].
A positive cytology may be predictive of a poorer outcome in patients with neoplastic pericardial disease. In an observational study of patients with cancer-related pericardial effusion, positive cytology was associated with decreased overall survival (median 7 versus 30 weeks for normal cytology) [13]. Patients with positive cytology were also more likely to require repeat pericardiocentesis or surgical intervention. (See "Pericardial disease associated with cancer: Management", section on 'Prevention and treatment of recurrent malignant pericardial effusion'.)
Flow cytometry — For patients with a suspected hematologic malignancy, such as leukemia or lymphoma, flow cytometry of the pericardial fluid is an important adjunct to cytology [65,66]. In an observational study of 115 serous cavity effusions from patients with a variety of malignant diagnoses, the provisional cytopathologic diagnosis was altered by the results of flow cytometry in 18 cases (16 percent of the total). Among those 18 cases, the diagnosis changed from atypical/suspicious to benign in eight cases and from benign or atypical/suspicious to malignant in 10 cases [66]. (See "Clinical presentation and initial evaluation of non-Hodgkin lymphoma", section on 'Pleural/pericardial fluid'.)
Indications for obtaining a pericardial specimen — For patients with negative cytology on pericardial effusion but for whom cancer is still highly suspected, we obtain a pericardial specimen for pathologic examination, since negative cytology does not exclude the diagnosis of cancer [67]. The pericardial specimen may be obtained using the following methods:
●Pericardial window – For patients who receive a pericardial window to facilitate drainage of pericardial fluid, the therapeutic drainage window created by a subxiphoid or transthoracic approach generally yields a >4 cm2 piece of tissue [68,69], which can be useful to confirm the diagnosis. The subxiphoid approach provides more limited exposure with resected pericardial specimens typically 2 to 4 cm in width and length. A transthoracic approach provides access to a larger region of pericardium (particularly on the left side where windows both anterior and posterior to the phrenic nerve can be created) enabling more reliable long-term drainage and a larger tissue specimen for analysis.
●Pericardial biopsy – For patients not receiving a pericardial window, a pericardial biopsy can be performed via a subxiphoid or transthoracic pericardiostomy (window). Pericardial specimens are generally at least 2 cm x 2 cm to enable adequate pericardial fluid drainage and a sufficient tissue specimen for pathologic analysis.
Constrictive pericarditis — Patients with constrictive pericarditis are unlikely to have undiagnosed cancer, as most are related to prior radiation therapy (RT). Pericardial cancer involvement is a rare cause of constrictive pericarditis that may be diagnosed by pathologic examination of pericardial biopsy or pericardiectomy specimens. (See 'Constrictive pericarditis' above.)
Acute pericarditis — In patients with cancer, causes of acute pericarditis include cancer-directed therapy (RT or antineoplastic agents such as immune checkpoint inhibitors), infection, and malignancy, as well as other causes of pericardial disease (table 2). (See 'Etiology' above and "Etiology of pericardial disease".)
For those with acute pericarditis and an associated significant pericardial effusion, pericardiocentesis can be performed and pericardial fluid removed for both diagnostic (ie, to distinguish malignancy from other causes) and therapeutic purposes. (See 'Pericardial effusion' above and "Acute pericarditis: Clinical presentation and diagnosis", section on 'Pericardiocentesis and pericardial biopsy'.)
For patients with suspected cancer who present with acute pericarditis alone (without effusion or mass), we do not routinely perform pericardial tissue sampling to diagnose cancer. It is extremely rare for malignancy to cause acute pericarditis without an associated pericardial effusion or mass, so the risks of this invasive procedure should be weighed against the likelihood of diagnosing malignancy. If cancer is still suspected after nondiagnostic or negative echocardiography, CT or CMR can be used to evaluate for potential signs of malignant pericardial involvement such as a loculated or hemorrhagic pericardial effusion or a pericardial or cardiac mass. (See 'Further cardiac imaging' above.)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Pericardial disease".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Beyond the Basics topic (see "Patient education: Pericarditis (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
●General principles
•Epidemiology – Pericardial disease occurs in approximately 10 to 20 percent of patients with cancer. (See 'Epidemiology' above.)
•Causes – Clinical conditions that cause pericardial disease in patients with cancer include malignancy, cancer therapy (eg, radiation therapy [RT], antineoplastic agents), and infection. (See 'Etiology' above.)
•Associated cancers – Among patients with cancer and pericardial effusion, the frequency of a malignant effusion is approximately 40 to 60 percent. Lung cancer is the most common malignancy to involve the pericardium. Other common cancers include breast, esophagus, melanoma, lymphoma, and leukemia. (See 'Malignancy' above.)
●Clinical presentation – In patients with cancer, pericardial disease can present as acute pericarditis, pericardial effusion with or without cardiac tamponade, constrictive pericarditis, or effusive-constrictive pericarditis. (See 'Clinical presentation' above.)
●When to suspect malignant pericardial disease in patients without previously known cancer – Patients who should undergo a detailed evaluation for occult cancer include those with persistent or recurrent pericarditis that is unresponsive to antiinflammatory therapy, those in whom cancer is suspected (eg, unexplained weight loss or a heavy smoking history), and those who present with a new large pericardial effusion or cardiac tamponade. (See 'General approach' above.)
●Diagnostic evaluation for pericardial disease
•Initial evaluation – Patients with suspected malignant pericardial disease are initially evaluated by history, physical examination, ECG, chest radiograph, and transthoracic echocardiogram. (See 'Initial evaluation' above.)
•Laboratory testing – In patients with suspected acute pericarditis, certain markers of inflammation (white blood cell count, erythrocyte sedimentation rate, and serum C-reactive protein) are often elevated, whether or not malignant pericardial disease is present. (See 'Laboratory tests' above and "Acute pericarditis: Clinical presentation and diagnosis", section on 'Our approach to diagnostic testing'.)
The approach to laboratory testing for suspected pericardial effusion and constrictive pericarditis are discussed separately. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Initial tests' and "Pericardial effusion: Approach to diagnosis", section on 'Laboratory tests'.)
•Further cardiac imaging – Cardiac computed tomography (CT) or cardiovascular magnetic resonance imaging (CMR) is obtained if initial testing, including echocardiography, is nondiagnostic, but pericardial disease is still suspected. Indications include a loculated or hemorrhagic pericardial effusion, constrictive pericarditis, or a pericardial mass. (See 'Further cardiac imaging' above.)
•Cardiac catheterization – Cardiac catheterization may be obtained if initial testing, including echocardiography, is inconclusive or equivocal for identifying cardiac tamponade or constrictive physiology (constrictive pericarditis versus restrictive cardiomyopathy). Cardiac catheterization is also used to monitor intracardiac pressures during pericardiocentesis.
●Diagnosis of malignant pericardial disease – The diagnosis of malignant pericardial disease is typically confirmed on pathology.
•Pericardial effusion – In patients with pericardial effusion of uncertain etiology who have undergone pericardial fluid sampling, cytologic examination is an initial step in identifying a malignant cause. Flow cytometry is an important adjunct to cytology for patients with a suspected leukemia or lymphoma. (See 'Cytology' above and 'Flow cytometry' above.)
For patients with negative cytology but for whom cancer is still highly suspected, we obtain a pericardial specimen for pathologic examination since negative cytology does not exclude the diagnosis of cancer. The pericardial specimen may be obtained during the creation of a pericardial window or through pericardial biopsy. (See 'Indications for obtaining a pericardial specimen' above.)
•Constrictive pericarditis – For the rare patient with constrictive pericarditis where cancer is the suspected etiology despite a thorough initial evaluation, the diagnosis is confirmed by pathologic examination of pericardial tissue. (See 'Constrictive pericarditis' above.)
•Acute pericarditis - For patients with acute pericarditis and an associated significant pericardial effusion, pericardiocentesis can be performed and pericardial fluid removed for both diagnostic (to distinguish malignancy from other causes) and therapeutic purposes.
For patients with acute pericarditis alone (without pericardial effusion), we do not routinely perform pericardial tissue sampling to diagnose cancer. If cancer is still suspected after nondiagnostic or negative echocardiography, CT or CMR can be obtained to evaluate for potential signs of malignant pericardial involvement such as a loculated or hemorrhagic pericardial effusion, or a pericardial or cardiac mass. (See 'Acute pericarditis' above.)
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Howard (Jack) West, MD, who contributed to earlier versions of this topic review.
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