Perivascular epithelioid clear cell tumor of the common bile duct

The American Journal of Surgical Pathology
Saha SadeghiHoracio Maluf

Abstract

The perivascular epithelioid clear cell tumor (PEComa) has been described in a number of locations, including the pancreas, uterus, bladder, prostate, and gastrointestinal tract. We report the existence of a similar tumor occurring in the distal common bile duct of a 51-year-old man admitted for obstructive jaundice. The tumor had characteristic histologic features of a PEComa, including a richly vascular organoid architecture, tumor cells with clear to lightly eosinophilic cytoplasm, and variably prominent nucleoli. Immunohistochemically, the tumor cells were positive for HMB-45 and neuron specific enolase but negative for epithelial markers, smooth muscle markers, other neuroendocrine markers, vimentin, melan-A, and S-100 protein. PEComas appear to be ubiquitous tumors with characteristic histology and immunophenotype. Although most of these tumors have behaved in a benign fashion, they should be considered tumors of uncertain malignant potential given previous reports of recurrence and metastases. During a short follow-up period following a conservative local excision, our patient remains free of disease.

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Citations

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Aug 2, 2006·Archives of Pathology & Laboratory Medicine·Jeremy R ParfittBret M Wehrli

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