KSHV/HHV8-Associated Extracavitary Primary Effusion Lymphoma Presenting as Multiple Lymphomatous Polyposis

Publication date: Available online 2 March 2018
Source:Human Pathology
Author(s): M.D Cyrus Oster, M.D Theodore Stein, M.D Sumire Kitahara, M.D Serhan Alkan, Qin Huang
Primary effusion lymphoma (PEL) is a distinct clinicopathologic entity usually characterized by presentation as a lymphomatous body cavity effusion in the absence of solid tumor mass or dissemination during its clinical course. PEL can also rarely occur as a solid lymphoma involving nodal and extranodal sites and is referred to as extracavitary PEL. Here we report a unique case of extracavitary PEL in a 49year-old HIV-seropositive patient, who presented with vague abdominal pain and 20 pound weight loss. Esophagogastroduodenoscopy and colonoscopy revealed >100 broad based intestinal polyps ranging from 2 mm to 3cm in size, spreading from the duodenum to the rectum as a typical impression of "intestinal polyposis syndrome". Multiple biopsies demonstrated sheets of large lymphoid cells with characteristic features of extracavitary PEL with strong KSHV/HHV8 virus positivity by immunohistochemistry. Extracavitary PEL presenting as distinctive multiple lymphomatous polyposis as manifested in the case has not been described previously.



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