{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:44:56Z","timestamp":1786977896731,"version":"build-2736575974"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,11,25]],"date-time":"2011-11-25T00:00:00Z","timestamp":1322179200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Familial adenomatous polyposis (FAP) is an autosomal dominant cancer predisposition syndrome that accounts for approximately 0.5\u20131% of all colorectal cancer cases. It is caused by germline mutations in the gene encoding the adenomatous polyposis coli (\n                    <jats:italic>APC<\/jats:italic>\n                    ) tumor suppressor. Somatic\n                    <jats:italic>APC<\/jats:italic>\n                    inactivation due to mutation or loss of heterozygosity (LOH) promotes the development of adenomatous polyps by stabilizing the transcriptional coactivator \u03b2\u2010catenin. 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\n                    <jats:italic>APC<\/jats:italic>\n                    mutation, strongly supporting a causative role of\n                    <jats:italic>APC<\/jats:italic>\n                    inactivation in their pathogenesis. Here we describe a 47\u2010year\u2010old 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 \u03b2\u2010catenin staining consistent with somatic\n                    <jats:italic>APC<\/jats:italic>\n                    inactivation, and molecular analysis confirmed biallelic\n                    <jats:italic>APC<\/jats:italic>\n                    inactivation in the tumor. Our findings provide the first evidence that ovarian steroid cell tumors may be an extracolonic manifestation of FAP and implicate \u03b2\u2010catenin activation as an oncogenic mechanism in ovarian steroid cell tumorigenesis. \u00a9 2011 Wiley Periodicals, Inc.\n                  <\/jats:p>","DOI":"10.1002\/gcc.20953","type":"journal-article","created":{"date-parts":[[2011,11,25]],"date-time":"2011-11-25T05:00:26Z","timestamp":1322197226000},"page":"283-289","source":"Crossref","is-referenced-by-count":16,"title":["Ovarian steroid cell tumor with biallelic adenomatous polyposis coli inactivation in a patient with familial adenomatous polyposis"],"prefix":"10.1002","volume":"51","author":[{"given":"Patrick J.","family":"Hu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stewart M.","family":"Knoepp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rong","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen R.","family":"Cho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,11,25]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64588-9"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63008-8"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.03485"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02054051"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ssu.2980100311"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20071"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-05-1024"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2010-1885"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800977"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.30.2810"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-pathol-011110-130235"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1080\/003655299750025741"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64045-X"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-10-1497"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(81)90021-0"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)61155-8"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-4-22"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199503303321302"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-198711000-00002"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/jjco\/hyp093"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.19.10330"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.275.5307.1784"},{"key":"e_1_2_5_24_1","first-page":"5007","article-title":"Biallelic inactivation of the APC gene in hepatoblastoma","volume":"55","author":"Kurahashi H","year":"1995","journal-title":"Cancer Res"},{"key":"e_1_2_5_25_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2008.080475"},{"key":"e_1_2_5_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02050923"},{"key":"e_1_2_5_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64821-3"},{"key":"e_1_2_5_28_1","first-page":"3011","article-title":"Characteristics of somatic mutation of the adenomatous polyposis coli gene in colorectal tumors","volume":"54","author":"Miyaki M","year":"1994","journal-title":"Cancer Res"},{"key":"e_1_2_5_29_1","first-page":"5079","article-title":"Coexistence of somatic and germ\u2010line mutations of APC gene in desmoid tumors from patients with familial adenomatous polyposis","volume":"53","author":"Miyaki M","year":"1993","journal-title":"Cancer 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