Malignant Pleural and Pericardial Effusions

MALIGNANT PERICARDIAL EFFUSION

Malignancy is one of the most common causes of pericardial effusion in the Western world.³⁴ Pericardial effusion in patients with current or past history of malignancy can be directly caused by the malignancy itself (contiguous extension or metastasis to the pericardium evidenced by identification of cancer cells in the pericardial fluid and/or in the pericardium) or due to inflammatory process such as benign idiopathic pericarditis or radiation-induced pericarditis.³⁴ Primary cancers of the pericardium, such as mesothelioma, fibrosarcoma, and lymphangioma, are exceedingly rare. Patients with malignant pericardial disease may be asymptomatic or present with a number of manifestations, with pericardial effusion being the most common. Cardiac tamponade due to malignant pericardial effusion (MPCE) accounts for at least 50% of all reported cases of pericardial fluid that require intervention. Only 15% to 25% of patients with documented metastasis to the pericardium have pericardial effusion and only a small percentage of those patients will develop pericardial tamponade.³⁴ Similar to MPEs, MPCEs are frequently indicative of advanced incurable malignancy, and the overall median survival of patients with MPCE is often <6 months.

Clinical Presentation

In most cases, MPCE is observed in patients with an established diagnosis of cancer, typically at late stages of their disease. MPCE is rarely seen as the initial manifestation of extracardiac malignancy. Nonspecific symptoms are frequent with MPCE; as such, pericardial effusion may remain unsuspected in patients for whom nonspecific symptoms are otherwise attributed to overall disease progression. Whether cardiac tamponade presents as the initial manifestation of MPCE is highly variable and depends on the rate of fluid accumulation, volume of pericardial fluid, and the patient’s underlying cardiac function. Classic signs and symptoms of cardiac tamponade include dyspnea, orthopnea, low cardiac output (peripheral vasoconstriction, cold clammy extremities, poor capillary refill, and diaphoresis), jugular venous distention, distant heart sounds, pulsus paradoxus, and narrowed pulse pressure. An electrocardiogram may show low-voltage complexes across all monitoring leads and electrical alternans.

Diagnostic Modalities

Radiographic and Echocardiographic Studies

Pericardial effusion should be suspected in the asymptomatic patient with cancer when an enlarged globular water-bottle pericardial silhouette is found on plain posteroanterior and lateral chest radiographs or more commonly detected by thoracic CT scans. Once a pericardial effusion is suspected, echocardiography should be performed to confirm its presence, to assess the hemodynamic significance of pericardial effusion, and to determine the presence of pericardial or intracardiac masses. Right atrial and ventricular collapse are the classic echocardiographic signs of cardiac tamponade, with sensitivity ranging from 38% to 60% and specificity ranging from 50% to 100%.³⁵ Echocardiography-guided pericardiocentesis is a preferred initial approach to alleviate pericardial tamponade.

Cytopathology and Histopathology

The cytology of a pericardial effusion determines definitively its benign or malignant nature. Malignant cells are identified in pericardial fluid from 66% to 79% of MPCE, with the cytology correlating with histologic diagnosis of the underlying malignancy in 100% of these individuals.³⁶ In contrast, parietal pericardial biopsy is frequently nondiagnostic because the distribution of malignant involvement is not uniform.

Treatment

General goals of treatment for MPCE include relief of immediate symptoms, confirmation of the malignant nature of the fluid, and prevention of recurrence. Therapy for MPCE should be tailored to the performance status and prognosis of each patient. Although simple pericardiocentesis can be life-saving in cases of cardiac tamponade, more definitive treatment options for clinically significant MPCE are indicated to minimize the risk of recurrence. These include surgical procedures such as subxiphoid pericardiostomy (pericardial window), transthoracic pericardial window or pericardiectomy (either by VATS or thoracotomy), and medical interventions such as percutaneous tube pericardiostomy with or without intrapericardial instillation of sclerosing agents.

Pericardiocentesis

Pericardiocentesis is the intervention of choice for patients with hemodynamic instability due to pericardial effusion as removal of as little as 50 ml of pericardial fluid can significantly improve signs and symptoms of acute cardiac tamponade. In patients with cancer with symptomatic pericardial effusion, pericardiocentesis may be performed to stabilize the patient prior to additional drainage procedures, such as percutaneous tube pericardiostomy or subxiphoid pericardial window. Echocardiography-guided percardiocentesis is the preferred technique as it minimizes complications and improves the success of the pericardiocentesis by delineating the size and location of the effusion relative to cardiac structures. Overall rates of complication and success are approximately 2.4% and 100%, respectively, for echocardiography-guided pericardial drainage as compared with 4.8% and 90%, respectively, for unassisted pericardiocentesis.³⁷

Percutaneous Tube Pericardiostomy and Pericardial Sclerotherapy

Following pericardiocentesis, the rate of fluid reaccumulation has been reported to range from 44% to 70%.³⁸ To improve these results, a 9-Fr pigtail draining catheter is now routinely placed into the pericardial space using Seldinger technique, following successful needle pericardiocentesis, to enable more complete evacuation of the effusion and provide access for sclerotherapy. Tetracycline and doxycycline have been most extensively evaluated as pericardial sclerosing agents. Maher and colleagues³⁸ reported 93 patients with MPCE treated by percutaneous pericardial drainage followed by tetracycline or doxycycline sclerosis. Successful placement of the pericardiostomy tube was achieved in 85 patients (92%). Pericardial effusion was controlled in 75 patients (88%); 10 patients (12%) did not respond to sclerosis, 8 of whom subsequently underwent surgical pericardiostomy. Successful sclerotherapy often requires multiple instillations of tetracycline or doxycycline (range, 1 to 8; median, 3); 50 patients required three or more instillations to control their effusions. Treatment-related complications (in decreasing order of frequency) included pain, catheter occlusion, fever, and atrial arrhythmias. The favorable results of this minimally invasive treatment strategy are offset by the need for repeated instillations of the sclerosing agent in order to achieve pericardial symphysis. Liu and associates³⁹ conducted a prospective study to evaluate the efficacy and toxicity of bleomycin versus doxycycline as sclerosing agents in MPCE. Bleomycin was found to be as effective as doxycycline in achieving satisfactory control of MPCE but with much less retrosternal pain. As a result, these authors recommend that bleomycin be considered the first-line chemical sclerosing agent for MPCE. Phase 2 random-assignment clinical trial evaluating intrapericardial bleomycin sclerotherapy following pericardial drainage (tube periocardiostomy or subxiphoid periocardiostomy) in 79 patients with lung cancer with MPCE only demonstrated a very modest additional clinical benefit of reducing recurrence of pericardial effusion.⁴⁰ Pericardial sclerotherapy, while being reported in these small series, is not a commonly performed procedure.

Percutaneous Balloon-Tube Pericardiostomy

Percutaneous balloon-tube pericardiostomy is an extension of the more commonly performed percutaneous tube pericardiostomy. In balloon-tube pericardiostomy, pericardiocentesis is performed as previously described but 150 to 200 ml of fluid is intentionally left in the pericardial space. Subsequently, dilatation of the needle tract is performed under fluoroscopy using a balloon catheter creating a larger pericardial opening to the subcutaneous space and mimicking surgical pericardiostomy to reduce recurrence. Ziskind and colleagues⁴¹

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