Extracavitary/solid variant of primary effusion lymphoma. Ann Diagn Pathol 2012 Dec;16(6):441-6
Date
04/14/2012Pubmed ID
22497739DOI
10.1016/j.anndiagpath.2012.03.004Scopus ID
2-s2.0-84868517175 (requires institutional sign-in at Scopus site) 52 CitationsAbstract
Primary effusion lymphoma (PEL) is a distinct clinicopathologic entity associated with human herpesvirus 8 (HHV8) infection that mostly affects patients with immunodeficiency. Primary effusion lymphoma usually presents as a malignant effusion involving the pleural, peritoneal, and/or pericardial cavities without a tumor mass. Rare cases of HHV8-positive lymphoma with features similar to PEL can present as tumor masses in the absence of cavity effusions and are considered to represent an extracavitary or solid variant of PEL. Here, we report 3 cases of extracavitary PEL arising in human immunodeficiency virus-infected men. Two patients had lymphadenopathy and underwent lymph node biopsy. One patient had a mass involving the ileum and ascending colon. In lymph nodes, the tumor was predominantly sinusoidal. The tumor involving the ileum and ascending colon presented as 2 masses, 12.5 × 10.6 × 2.6 cm in the colon and 3.6 × 2.7 × 1.9 cm in the ileum. In each case, the neoplasms were composed of large anaplastic cells, and 2 cases had "hallmark cells." Immunohistochemistry showed that all cases were positive for HHV8 and CD138. One case also expressed CD4 and CD30, and 1 case was positive for Epstein-Barr virus-encoded RNA. Evidence of B-cell differentiation was poorly developed in all tumors. These cases highlight the importance of assessing HHV8 in an anaplastic tumor that arises in a human immunodeficiency virus-positive patient and further contributes to the limited literature currently available for extracavitary PEL.
Author List
Kim Y, Leventaki V, Bhaijee F, Jackson CC, Medeiros LJ, Vega FMESH terms used to index this publication - Major topics in bold
AdultBiomarkers, Tumor
Biopsy
Colon, Ascending
Diagnosis, Differential
Epstein-Barr Virus Infections
HIV
HIV Infections
HIV Seropositivity
Herpesvirus 4, Human
Herpesvirus 8, Human
Humans
Ileum
Immunophenotyping
Lymph Nodes
Lymphoma, Large-Cell, Anaplastic
Lymphoma, Primary Effusion
Male
Middle Aged