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

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 i...

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Published inPathology international Vol. 56; no. 1; pp. 46 - 50
Main Authors Yamamoto, Hidetaka, Oda, Yoshinao, Yao, Takashi, Oiwa, Toshio, Kobayashi, Chikashi, Tamiya, Sadafumi, Kawaguchi, Ken‐ichi, Hino, Okio, Tsuneyoshi, Masazumi
Format Journal Article
LanguageEnglish
Published Melbourne, Australia Blackwell Science Pty 01.01.2006
Subjects
Adult
Biomarkers, Tumor - analysis
Carcinoma - metabolism
Carcinoma - pathology
colon
Colonic Neoplasms - metabolism
Colonic Neoplasms - pathology
cyclin D1
Female
Follow-Up Studies
HMB‐45
Humans
Immunohistochemistry
LOH
malignant
PEComa
perivascular epithelioid cell
Treatment Outcome
TSC1
TSC2
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:1320-5463
1440-1827
DOI:10.1111/j.1440-1827.2006.01917.x