Extracavitary primary effusion lymphoma presenting as a cutaneous tumor: a case report and literature review.

[primary effusion lymphoma]

Primary effusion lymphoma is an unusual form of aggressive B-cell lymphoma universally associated with human herpesvirus 8 (HHV 8 ) that involves mostly human immunodeficiency virus (HIV )-infected patients . Characteristically , it presents as a malignant serous effusion involving body cavities , but without associated tumor mass . Exceptionally , HHV 8 -positive lymphomas with features identical to primary effusion lymphoma may present as mass lesions in the absence of cavity effusions along the course of the disease , and are regarded as extracavitary or solid variants of the disorder . These rare forms are extremely rare in the skin . We report a case of extracavitary primary effusion lymphoma arising in a HIV-infected male , who presented with two subcutaneous masses involving the skin of the abdominal and inguinal regions as the first manifestation of the process . Kaposi sarcoma was not present in the skin surface or mucous membranes . Extensive studies failed to demonstrate involvement of other organs and the case was considered as an example of extracavitary primary effusion lymphoma originating primarily in the skin . Herein , we review the few reported cases of solid primary effusion lymphoma involving the skin in order to delineate the clinicopathologic , immunohistochemical and molecular characteristics of this rare lymphoma in the skin .