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Ovarian Steroid Cell Tumor with Biallelic Adenomatous Polyposis Coli Inactivation in a Patient with Familial Adenomatous Polyposis by Patrick J. Hu; Stewart M. Knoepp; Rong Wu; Kathleen R. Cho is a Medicine article available to read on EtoBox.

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## Abstract Familial adenomatous polyposis (FAP) is an autosomal dominant cancer predisposition syndrome that accounts for approximately 0.5–1% of all colorectal cancer cases. It is caused by germline mutations in the gene encoding the adenomatous polyposis coli (__APC__) tumor suppressor. Somatic __APC__ inactivation due to mutation or loss of heterozygosity (LOH) promotes the development of adenomatous polyps by stabilizing the transcriptional coactivator β‐catenin. Although colorectal cancer is by far the most common malignancy seen in FAP patients, the widespread use of prophylactic colectomy in these patients has increased the clinical importance of extracolonic tumors that are part of the neoplastic spectrum in FAP. Many of these tumors exhibit LOH or somatic __APC__ mutation, strongly supporting a causative role of __APC__ inactivation in their pathogenesis. Here we describe a 47‐year‐old female FAP patient with clinical manifestations of virilization who was found to have an ovarian steroid cell tumor, a rare neoplasm not known to be associated with FAP. Immunohistochemical analysis of the ovarian tumor demonstrated strong nuclear β‐catenin staining consistent with somatic

Who reads Ovarian Steroid Cell Tumor with Biallelic Adenomatous Polyposis Coli Inactivation in a Patient with Familial Adenomatous Polyposis?

Author

Patrick J. Hu; Stewart M. Knoepp; Rong Wu; Kathleen R. Cho

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); Wiley-Liss Inc; Wiley (ISSN 1045-2257)

Published

2011

Language

EN

Field

Medicine (Life Sciences)