A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma-A Case Report and Review of the Literature.

[primary effusion lymphoma]

Primary effusion lymphoma (PEL ) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion (s ) without any nodal enlargement or tumor masses . It belongs to the group of AIDS related non-Hodgkin 's lymphomas . First described in 1996 in HIV infected individuals who were coinfected with Kaposi 's sarcoma -associated herpesvirus (KSHV ) or HHV-8 virus , it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001 . The definition included association with HHV-8 virus as a mandatory diagnostic criterion . However , cases were later reported where PEL -like disease process was diagnosed in HHV-8 negative patients . This was eventually recognized as a rare but distinct entity termed as "HHV-8 -unrelated PEL -like lymphoma ". Herein , we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity . Till date , only around 50 cases of HHV-8 -unrelated PEL -like lymphoma have been reported and our case being EBV , HIV , and Hepatitis C negative makes it very unique and rare occurrence . We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy .

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Diseases presenting "tumor masses" symptom

cushing syndrome

primary effusion lymphoma

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