Intravascular extranodal NK/T-cell lymphoma, nasal type, presenting as acute abdomen

Abstract

Intravascular lymphomas (IVLs) overwhelmingly express B-cell phenotypes, although T lineage is not uncommon. However, purely intravascular NK/T-cell lymphomas are vanishingly rare. We present a case of intravascular extranodal NK/T-cell lymphoma (ENKTCL), nasal type, in a 37-year-old Hispanic man admitted for severe abdominal pain. Intraoperative evaluation revealed necrotic small bowel and multiple mesenteric venous thromboses. Initial histologic examination demonstrated classic changes of ischemic bowel without evidence of dense lymphoid infiltrates or other mass lesions. However closer review of the mesenteric vasculature exposed an angioinvasive and angiodestructive process comprised of lymphomatous cells which were positive by immunohistochemistry for CD2, CD3, CD8, BCL2, TIA-1, Granzyme B, and Epstein-Barr virus (EBV) EBER in situ hybridization. Although exceedingly rare, isolated intravascular ENKTCL, nasal type, may present without a mass; such lesions must be recognized since IVLs typically exhibit widespread dissemination and portend a dire prognosis with most patients inevitably succumbing to their disease.