Neoplastic Pericardial Disease BY Prof. A. Frere MD Cardiology Zagazig- Egypt

Epidemiology The pericardium is a common target for neoplastic involvement, usually manifest as pericardial effusion, cardiac tamponade, or constrictive pericarditis Among patients with acute pericardial disease majority have idiopathic or viral pericarditis 6% have malignant etiology in reported series Am J Cardiol 1995; 75:378

Epidemiology Attempts to make a specific diagnosis are generally limited to patients with persistent pericarditis unresponsive to antiinflammatories cardiac tamponade a new large pericardial effusion Among such patients in whom a diagnosis is established, malignancy has accounted for 25 to 40 percent of cases Am J Med 1993; 95:209

PERICARDIAL DISEASE IN PATIENTS WITH KNOWN MALIGNANCY Several neoplasms metastasize or spread either directly or, more commonly, via lymphatics to the pericardium The most common of these neoplasms are carcinomas of the lung or breast and the lymphomas Primary pericardial neoplasia, particularly mesothelioma, hemangioma, or angiosarcoma, is seen much less frequently

MYOCARDIAL DISEASE IN PATIENTS WITH KNOWN MALIGNANCY Most pericardial tumors do not invade the myocardium However, myocardial involvement is a classic feature of melanoma and is common in leukemia Myocardial neoplasia is usually silent and discovered only at autopsy or, less often, during surgery

Pericardial Effusion in Patients with Known Malignancy Malignant pericardial effusion is often asymptomatic and discovered by imaging or at autopsy Once effusion becomes clinically apparent, morbidity and mortality are high, since many symptomatic patients have cardiac tamponade and constriction Patients with constrictive pericarditis due to malignancy are unlikely to be candidates for radical pericardiectomy

Cytology and pericardial biopsy Most patients with malignant pericardial disease are known to have a malignant tumor before evidence of pericardial involvement develops Cytologic analysis and pericardial biopsy may disclose neoplasm in those cases in which pericardiopathy is the presenting manifestation of distant neoplasia

Cytology and pericardial biopsy Sensitivity of cytology for malignant pericardial disease varies widely (eg, 67-90 %). Causes of false-negative cytology include scant cellularity the obscuring effect of blood the absence of an expert exfoliative cytologist sampling errors in pericardial biopsy A negative cytology alone should not exclude the diagnosis of malignant disease.

Imaging studies Echocardiography CT and MRI the primary imaging tool to establish pericardial effusion useful to quantify the volume of the effusion and to evaluate its hemodynamic effects (tamponade or constrictive pericarditis) CT and MRI provide excellent information for loculated pericardial effusion both are superior to echocardiography for determining whether an effusion is a transudate, exudate, or blood effusion large tumor masses in the pericardium can be recognized by all of the imaging studies

Nonmalignant pericardial effusion Patients with neoplasia can also develop nonmalignant pericardial disease In two series of 241 patients with pericardial effusion and known malignancy, 45 to 60 % could not be shown to have neoplastic pericardial disease; a cause could be established in only one-quarter of these patients Potential causes include radiation, infection related to treatment unrelated disorders: uremia, collagen vascular disease, and acute viral or idiopathic pericarditis that responds quickly to treatment with an antiinflammatory drug Cancer 1995; 76:1377 Cardiothorac Surg 1999; 16:287

Nonmalignant pericardial effusion false-negative results should be suspected when the effusion does not behave in the predicted way In patients with systemic signs such as weight loss or persistent cough abdominal pain or dyspepsia a palpable mass in the breast or abdomen abnormal opacity on CT, ultrasound, or chest radiogram cytological is most useful when an expert exfoliative cytologist is available

Recommendations for pericardiocentesis or pericardial biopsy In the absence of clues to the presence of malignant pericardial disease or clinical tamponade or constriction pericardiocentesis or pericardial biopsy should be performed only in patients with persistent or progressive disease that is unresponsive to antiinflammatory therapy Evaluation is also probably indicated for a new, large pericardial effusion

Treatment tamponade and compressive syndrome Early tamponade with only mild hemodynamic compromise may be treated conservatively with careful monitoring repeated echocardiography avoidance of volume depletion, and therapy aimed at the underlying malignancy To avoid missing effusive constrictive pericarditis, it is important to monitor right heart pressures before and after performing pericardiocentesis

Treatment asymptomatic effusion Asymptomatic pericardial effusion associated with cancer does not require require therapy Elective therapy include Prolonged catheter drainage Substernal pericardiotomy Limited or radical pericardiectomy Obliteration of the pericardial sac by intrapericardial injection of a sclerosing agent Mayo Clin Proc 2000; 75:248

Treatment prolonged catheter drainage The treatment of choice simple, relatively atraumatic, safe, and effective Intermittent, not continuous, drainage is preferred drainage catheter may need to remain in place for several days until drainage is  20 - 30 ml/24 hrs the benefit favors its retention over the slight increased risk of infection to a considerable extent, it has obviated the need for sclerosing agent or pericardiotomy

Treatment balloon pericardiotomy balloon pericardiotomy can be performed with low risk and often under local anesthesia, an important consideration for these patients in whom the quality of remaining life is paramount However, the procedure is usually painful given the choice, patients may prefer surgical pericardiotomy J Am Coll Cardiol 1993; 21:1

Treatment substernal pericardiotomy some physician use this method as initial treatment majority employ it in cases of continuous (>4 days) copious drainage; a relatively infrequent problem Prolonged catheter drainage and limited pericardiotomy are generally palliative measures in patients with a short expected survival

Treatment surgical pericardiotomy For patients with the possibility of prolonged survival in whom the effusion recurs or progressively increases in size, surgical pericardiectomy is the definitive procedure with the intrapericardial injection of agents

Treatment sclerosing therapy use of this approach is declining sclerosing agents (such as tetracycline) generally have good results but are very painful intrapericardial lidocaine should be used first with prolonged survival, sclerosis may cause constrictive pericarditis other compounds include cisplatin, thiotepa, carboplatin, OK432, interferon alfa-2b; discuss with an oncologist when using these agents Hematol Oncol Clin North Am 1997; 11:253

PRIMARY PERICARDIAL NEOPLASIA The most common are: Mesothelioma, which may be benign, but is more frequently highly malignant Sarcoma, particularly angiosarcoma Hemangiomata, which are benign but can cause pericardial effusion Intrapericardial pheochromocytoma which usually is associated with symptoms from catecholamine excess Teratomata, neuroblastoma, and thymoma primarily occur in infants and children and may be benign or malignant

Am Heart J 1992; 124:802 Br Heart J 1987; 57:54 Mesothelioma Although exceedingly rare, it is one of the most common primary malignant pericardial tumors association with asbestos exposure is well established It is important to diagnose because treatment can yield a year or more of good quality life It most often arises in young or middle aged adults and may present with pericardial effusion, including tamponade or constrictive pericarditis Am Heart J 1992; 124:802 Br Heart J 1987; 57:54

Mesothelioma MRI is most accurate when there is a discrete mass Mesothelioma more often envelops the heart and causes rapidly progressive constrictive pericarditis may be difficult to distinguish from pericardial constriction of other causes, such as tuberculous pericarditis

Lipoma Typically insignificant, but can assume gigantic size May be mistaken for a huge pericardial effusion or massive cardiomegaly The tumor may infiltrate the myocardium. When the ventricular septum is invaded, the pericardial and right ventricular cavity may communicate.

Pericardial (springwater) cyst benign and almost always congenital commonly located in the right cardiophrenic angle appears as a perfectly round, fluid density, usually 2 to 4 cm in diameter, filled with clear liquid rarely, a pericardial cyst may calcify or rupture secure diagnosis can be made with CT or MRI no treatment is required Acquired pericardial cysts are exceedingly rare - seen with neoplastic, parasitic, or traumatic disease Chest 1971; 60:611