Colonic angiomyolipoma with a monotypic expression and a predominant epithelioid component

Queens Medical Centre University Hospital, Nottingham NG7 2UH, UK.
Journal of Clinical Pathology (Impact Factor: 2.92). 11/2005; 58(10):1107-9. DOI: 10.1136/jcp.2004.025130
Source: PubMed


Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas/PEComas reported in the literature, this case formed a large, mainly extrinsic mass and was monotypic, and composed entirely of the myomatous component with no adipose tissue or typical vasculature.

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Available from: Tom Mcculloch, Sep 04, 2014
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    ABSTRACT: Angiomyolipoma is apparently a part of a family of neoplasms that derive from perivascular epitheloid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon and the most common extrarenal site is the liver. Only two cases of adrenal angiomyolipoma are reported in English literature. Authors wish to add one more case to world literature. Because of its large size and symptomatic presentation of extremely rare tumor merits documentation.
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