SESSION TITLE: Cancer Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 27, 2014 at 11:00 AM - 12:00 PM
INTRODUCTION: A case of 28 yo male who presented with pericardial effusion and mediastinal mass and was diagnosed with primary pericardial mesothelioma (PPM) on pericardial biopsy. Patient did not respond to chemotherapy and died 3 months later.
CASE PRESENTATION: A 28 yo male presented with 2 weeks of progressive dyspnea and severe orthopnea precluding daily activities and limiting sleep. Physical examination revealed a young male sitting upright in moderate respiratory distress. Neck examination showed markedly elevated JVP. His heart sounds were distant with lungs clear to auscultation. There was no history of asbestos exposure. Transthoracic echocardiography revealed significant pericardial effusion. CT chest showed anterior mediastinal mass. Patient underwent pericardial window with pericardial biopsy. The results of the biopsy returned as malignant pericardial mesothelioma of epithelioid to spindled phenotype. CK 5/6, AE 1/3 and D2-40 stains were positive (Figure 1) while TTF1, CD5, P53 and CD20 were negative. Repeat transthoracic echocardiography 1 week later revealed persistent large circumferential pericardial effusion. CT showed encasement of the ascending aortic arch and pulmonary vessels by the conglomerate of mediastinal lymphadenopathy (Figure 2) due to which he was not deemed to be a surgical candidate. Palliative chemotherapy with Carboplatin/Pemetrexed was tried without significant response. Patient died 3 months after the diagnosis.
DISCUSSION: Among four types of malignant mesotheliomas pericardial is the rarest. The role of asbestos exposure in PPM remains unclear. Common clinical presentations are constrictive pericarditis, pericardial effusion, cardiac tamponade and congestive heart failure. Radical surgery remains the main stem of therapy in a localized cases, although it is often not possible due to involvement of the great vessels. PPM responds poorly to chemotherapy, although recently published study showed improved survival with combination of Premetrexed/Cisplatin.
CONCLUSIONS: PPM is an extremely rare malignancy with total of 200 cases described in the literature. The diagnosis is usually delayed due to insidious onset of non-specific symptoms. Surgical resection is the main stem of therapy for localized disease due to poor response of the tumor to systemic chemotherapy. The overall prognosis of PPM remains very poor with survival less than 6 months.
Reference #1: Primary malignant mesothelioma of the pericardium. Case report and literature review; Thomason, Schlegel; Tex Heart Inst J. 1994; 21(2)
DISCLOSURE: The following authors have nothing to disclose: Nina Zatikyan, Jay Peters, Muhammad Pathan
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