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

Primary effusion lymphoma is an unusual form of aggressive B‐cell lymphoma universally associated with human herpesvirus 8 (HHV8) 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, HHV8‐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.