Perivascular epithelioid clear cell tumor of the common bile duct.
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.
Sadeghi
Saha
S
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical School, 660 S. Euclid Avenue, St. Louis, MO 63110, USA.
Krigman
Hannah
H
Maluf
Horacio
H
D018262:Adenocarcinoma, Clear Cell / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D001650:Bile Duct Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001808:Blood Vessels / Q000473:pathology*; D003135:Common Bile Duct / Q000098:blood supply / Q000473:pathology* / Q000601:surgery; D018572:Disease-Free Survival; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011933:Reagent Kits, Diagnostic
D014408:Biomarkers, Tumor; D011933:Reagent Kits, Diagnostic