Malignant perivascular epithelioid cell tumor of the colon: report of a case with molecular analysis.

Abstract:

:Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm, and malignant cases are extremely rare. A case of malignant PEComa arising in the colon is described herein. The patient was a 43-year-old Japanese woman without a history of tuberous sclerosis complex. The tumor occurred in the abdominal cavity attached to the serosal side of the descending colon. Histologically, the tumor consisted of sheets or closely packed nests of epithelioid cells with clear or eosinophilic cytoplasms. The tumor cells were positive for HMB-45 but negative for S-100 protein and cytokeratins by immunohistochemical staining. Ki-67 labeling index was 2.9%. Peritoneal dissemination of tumor occurred at 20 months and the patient died of tumor at 38 months after the initial operation. This was considered to be a case of malignant PEComa, based on the histological and clinical features. Tumor cells showed overexpression of cyclin D1 but lacked the loss of heterozygosity of the TSC1 and TSC2 genes. The result suggests that the overexpression of cyclin D1 may play an important role in the tumorigenesis of PEComa. Because PEComas can behave in an aggressive manner, careful follow up is warranted.

journal_name

Pathol Int

journal_title

Pathology international

authors

Yamamoto H,Oda Y,Yao T,Oiwa T,Kobayashi C,Tamiya S,Kawaguchi K,Hino O,Tsuneyoshi M

doi

10.1111/j.1440-1827.2006.01917.x

subject

Has Abstract

pub_date

2006-01-01 00:00:00

pages

46-50

issue

1

eissn

1320-5463

issn

1440-1827

pii

PIN

journal_volume

56

pub_type

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