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10 December 2014 - Case of the Week #336

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Case of the Week #336

Clinical History:
A 42 year old man with HIV and Hepatitis B virus had six months of coughing / congestion. He underwent a video-assisted thoracoscopy with wedge biopsy

Micro images:


Additional images:


What is your diagnosis?































Diagnosis:
Kaposi sarcoma (multiple foci)

Discussion:
Kaposi sarcoma of the lung or soft tissue is a vascular proliferative disorder mediated by inflammatory cytokines and angiogenic growth factors in patients with HHV8 / KHSV (Kaposi sarcoma associated herpesvirus) infection, influenced by immune status. It may originate from a cell type capable of undergoing lymphatic differentiation, based on D2-40 staining (Mod Pathol 2002;15:434). It is usually associated with AIDS, but occasionally is present in HIV negative patients with other immunocompromise or organ transplant. Kaposi sarcoma is usually limited to skin, but may involve mucus membranes, lymph nodes and visceral organs such as the lung.

In the lung, radiologic and clinical findings include pleural effusion, solitary lung nodules, diffuse reticulonodular pulmonary infiltrates and peribronchovascular and interlobular septal thickening (Orphanet J Rare Dis 2009 Jul 14;4:18). Grossly, tumor follows lymphatic channels, and appears as discrete, dark red hemorrhagic nodules with growth along septa and infiltration of small airways, pulmonary arteries and veins.

In this case (reviewed by Dr. Thomas Colby, Mayo Clinic), the biopsy showed alveoli with foamy macrophages, proteinaceous material and inflammatory debris. However, no fungal elements or Pneumocystis organisms were identified on GMS / Grocott methenamine-silver staining (stain not shown). The nodules were composed of a fascicle-like pattern of mitotically active, pleomorphic spindle cells in a background of inflammatory cells and red blood cells. Small vessels and slitlike spaces with hyaline droplets are often present. The spindle cells are positive for CD34 and HHV8 (immunostains not shown). HHV8 immunostaining is also present in primary effusion lymphoma, multicentric Castleman disease and multicentric Castleman disease-associated plasmablastic lymphoma. In HIV+ patients, HHV8 may also be present in hemangiomas (Mod Pathol 2005;18:463).

Management consists of HAART / highly active antiretroviral therapy, followed by systemic chemotherapy (Mayo Clin Proc 2012;87:e77).




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and Shivani Thakore, Associate Medical Editor
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