From the archives of MD Anderson Cancer Center: Primary effusion lymphoma with simultaneous involvement of the retroperitoneum and pleural cavity

Primary effusion lymphoma (PEL) is a rare neoplasm that arises in the context of severe immunosuppression. Acquired immunodeficiency syndrome (AIDS) as a result of human immunodeficiency virus (HIV) infection is the most common cause of immunodeficiency in patients who develop PEL. These neoplasms usually involve one or more body cavities, so-called classic PEL. The pleural cavity is most often involved, followed by the peritoneal and pericardial cavities. Involvement of the cerebrospinal fluid (CSF) and meninges is rare. A subset of patients can present with a tissue-based mass, known as the extracavitary variant. We encountered a patient with HIV infection and severe immunosuppression who presented initially with mediastinal, retroperitoneal mass and bilateral pleural effusions. He subsequently developed CSF involvement. Despite therapy, the patient relapsed with chest wall disease 6 months later and died shortly thereafter. Our literature review yielded about 400 cases of PEL reported previously. About 65 % of PEL patients have had AIDS, but a subset of patients had immunosuppression attributable to organ transplantation or physiological immunosenescence. CSF involvement has been reported in ~2 % of patients, and about 10 % of patients had both body cavity and extracavitary disease. The pathologic findings in this case were typical of extracavitary PEL. The neoplastic cells had features of plasmablasts and were positive for HHV-8, Epstein-Barr virus encoded RNA (EBER) and plasma cell associated markers, and were negative for B-cell antigens. The prognosis of patients with PEL is usually poor with a median survival less than one year in most studies. We use this patient's case as an illustration of PEL and we review the clinicopathologic findings and differential diagnosis of PEL.