{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T05:54:14Z","timestamp":1648533254919},"reference-count":56,"publisher":"Walter de Gruyter GmbH","issue":"5-6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A 14-year-old female with classical congenital adrenal hyperplasia because of 21-hydroxylase deficiency underwent bilateral adrenalectomy at 6 years of age as a result of poor hormonal control. Because the patient was adrenalectomized, extra adrenal androgen production was suspected. Imaging studies including pelvic ultrasound and pelvic magnetic resonance imaging (MRI) were obtained to evaluate for adrenal rest tumors of the ovaries. Abdominal MRI was obtained to evaluate for residual adrenal tissue. A cystic lesion arising from her right ovary suspicious for ovarian neoplasm was noted on pelvic MRI. Right salpingo-oophorectomy was performed and histopathological examination revealed ovarian serous adenocarcinoma, low-grade, and well-differentiated. Tumor marker CA-125 was elevated and additional ovarian cancer staging workup confirmed stage IIIC due to one lymph node positive for carcinoma. The patient then developed a large left ovarian cyst, which led to a complete total abdominal hysterectomy and removal of the left ovary and fallopian tube. Pathology confirmed ovarian serous adenocarcinoma with microscopic focus of carcinoma in the left ovary. After numerous complications, the patient responded well to chemotherapy, CA-125 levels fell and no evidence of carcinoma was observed on subsequent imaging. To our knowledge, this is the first reported case of an ovarian serous adenocarcinoma in a patient with CAH. Although rare, we propose that the ovaries were the origin of androgen production and not residual adrenal tissue. The relationship between CAH and ovarian carcinomas has yet to be established, but further evaluation is needed given the poor survival rate of high-grade serous ovarian carcinoma.<\/jats:p>","DOI":"10.1515\/jpem-2014-0299","type":"journal-article","created":{"date-parts":[[2014,11,26]],"date-time":"2014-11-26T17:59:31Z","timestamp":1417024771000},"source":"Crossref","is-referenced-by-count":3,"title":["Ovarian carcinoma in a 14-year-old with classical salt-wasting congenital adrenal hyperplasia and bilateral adrenalectomy"],"prefix":"10.1515","volume":"28","author":[{"given":"Christian","family":"Pina","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmed","family":"Khattab","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip","family":"Katzman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauren","family":"Bruckner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey","family":"Andolina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"New","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mabel","family":"Yau","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref01","first-page":"29","article-title":"New Congenital adrenal hyperplasia owing to hydroxylase deficiency In New editors Genetic steroid disorders London","volume":"21","author":"Lekarev","year":"2014"},{"key":"ref431","article-title":"Average risks of breast and ovarian cancer associated with or mutations detected in case Series unselected for family a combined analysis of studies","volume":"22","author":"Antoniou","year":"2003","journal-title":"history Am J Hum Genet"},{"key":"ref311","first-page":"61","article-title":"Global cancer","author":"Jemal","year":"2011","journal-title":"statistics CA Cancer J Clin"},{"key":"ref451","doi-asserted-by":"crossref","first-page":"2084","DOI":"10.1210\/jc.2012-2298","article-title":"Use of PET with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia","author":"Crocker","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"key":"ref641","first-page":"61","article-title":"Global cancer","author":"Jemal","year":"2011","journal-title":"statistics CA Cancer J Clin"},{"key":"ref11","first-page":"866","article-title":"Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to hydroxylase deficiency","volume":"21","author":"Pang","year":"1988","journal-title":"Pediatrics"},{"key":"ref491","first-page":"109","article-title":"Bilateral Leydig cell tumor of the ovary in a woman with congenital adrenal hyperplasia The first reported case","volume":"20","author":"Luton","year":"1991","journal-title":"Presse Med"},{"key":"ref121","doi-asserted-by":"crossref","first-page":"2084","DOI":"10.1210\/jc.2012-2298","article-title":"Use of PET with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia","author":"Crocker","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"key":"ref211","first-page":"95","article-title":"Congenital adrenal hyperplasia due to steroid hydroxylase deficiency an clinical practice guideline","volume":"21","author":"Speiser","year":"2010","journal-title":"Endocrine Society J Clin Endocrinol Metab"},{"key":"ref391","doi-asserted-by":"crossref","first-page":"549","DOI":"10.3109\/09513590.2014.907260","article-title":"Woman with virilizing congenital adrenal hyperplasia and leydig cell tumor of the ovary","volume":"30","author":"Fern\u00e1ndez","year":"2014","journal-title":"Gynecol Endocrinol"},{"key":"ref191","first-page":"9","article-title":"disgerminoma gonadoblastoma Ovarian neoplasms in children and adolescents","author":"Motlik","year":"1973"},{"key":"ref471","article-title":"NM AR HM Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia : results of ultrasonography and MR imaging","volume":"14","author":"Stikkelbroeck","year":"2004"},{"key":"ref151","first-page":"339","article-title":"Del Ovarian adrenal - like tissue in congenital adrenal hyperplasia","author":"Russo","year":"1998","journal-title":"Engl J Med"},{"key":"ref331","first-page":"29","article-title":"New Congenital adrenal hyperplasia owing to hydroxylase deficiency In New editors Genetic steroid disorders London","volume":"21","author":"Lekarev","year":"2014"},{"key":"ref251","first-page":"274","article-title":"Serum gonadotropins and steroid hormones and the development of ovarian cancer","volume":"30","author":"Helzlsouer","year":"1995","journal-title":"J Am Med Assoc"},{"key":"ref531","first-page":"151","article-title":"giant ovarian cyst in a neonate with classical hydroxylase deficiency with very high testosterone levels demonstrating a high - dose hook effect","volume":"21","author":"G\u00fcran","year":"2012","journal-title":"Clin Res"},{"key":"ref601","first-page":"16","article-title":"Endogenous androgens and risk of epithelial ovarian cancer : results from the European Prospective Investigation into Cancer EPIC","volume":"23","author":"Rinaldi","year":"2007","journal-title":"Nutrition Cancer Epidemiol Biomarkers"},{"key":"ref231","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/S0090-8258(03)00407-4","article-title":"s syndrome due to ovarian serous adenocarcinoma secreting multiple endocrine substances : a case report and immunohistochemical analysis","author":"Suzuki","year":"2003","journal-title":"Gynecol Oncol"},{"key":"ref481","first-page":"339","article-title":"Del Ovarian adrenal - like tissue in congenital adrenal hyperplasia","author":"Russo","year":"1998","journal-title":"Engl J Med"},{"key":"ref101","article-title":"Average risks of breast and ovarian cancer associated with or mutations detected in case Series unselected for family a combined analysis of studies","volume":"22","author":"Antoniou","year":"2003","journal-title":"history Am J Hum Genet"},{"key":"ref401","doi-asserted-by":"crossref","DOI":"10.2169\/internalmedicine.50.5401","article-title":"woman with salt - wasting congenital adrenal hyperplasia presenting with a mucinous ovarian cystadenoma during pregnancy","author":"Kamata","year":"2011","journal-title":"Intern Med"},{"key":"ref411","first-page":"125","article-title":"CA the past the future Markers","volume":"13","author":"Bast","year":"1998","journal-title":"Int J Biol"},{"key":"ref361","first-page":"37","article-title":"High frequency of nonclassical steroid hydroxylase deficiency","volume":"21","author":"Speiser","year":"1985","journal-title":"Am J Genet"},{"key":"ref421","first-page":"706","article-title":"mutation frequencies and cancer penetrances : a kin - cohort study in Ontario","author":"Risch HA","year":"2006","journal-title":"Population Natl Cancer Inst"},{"key":"ref591","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1002\/ijc.10990","article-title":"Circulating levels of sex steroid hormones and risk of ovarian cancer","author":"Lukanova","year":"2003","journal-title":"Int J Cancer"},{"key":"ref51","first-page":"521","article-title":"Ovarian masses in children and adolescents an analysis of clinical cases","volume":"27","author":"Zhang","year":"2014","journal-title":"J Pediatr Gynecol"},{"key":"ref201","first-page":"151","article-title":"giant ovarian cyst in a neonate with classical hydroxylase deficiency with very high testosterone levels demonstrating a high - dose hook effect","volume":"21","author":"G\u00fcran","year":"2012","journal-title":"Clin Res"},{"key":"ref301","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1016\/j.ejca.2006.11.010","article-title":"the risk of epithelial ovarian cancer : a systematic meta - analysis","author":"Olsen","year":"2007","journal-title":"Obesity review Eur J Cancer"},{"key":"ref21","first-page":"1","article-title":"New - phenotype correlation in families with congenital adrenal hyperplasia owing to - hydroxylase deficiency","author":"Abraham","year":"2013","journal-title":"Proc Natl Acad Sci USA"},{"key":"ref181","doi-asserted-by":"crossref","first-page":"69","DOI":"10.4158\/EP13092.CR","article-title":"Bilateral ovary adrenal rest tumor in a congenital adrenal hyperplasia following adrenalectomy","volume":"20","author":"Zaarour","year":"2014","journal-title":"Endocr Pract"},{"key":"ref461","doi-asserted-by":"crossref","first-page":"4583","DOI":"10.1210\/jc.2007-0383","article-title":"Testicular adrenal rest tumor and cell function in boys with classical congenital adrenal hyperplasia","author":"Martinez","year":"2007","journal-title":"J Clin Endocrinol Metab"},{"key":"ref321","first-page":"5","article-title":"Ovarian serous carcinoma : recent concepts on its origin and","author":"Li","year":"2012","journal-title":"carcinogenesis Hematol Oncol"},{"key":"ref131","doi-asserted-by":"crossref","first-page":"4583","DOI":"10.1210\/jc.2007-0383","article-title":"Testicular adrenal rest tumor and cell function in boys with classical congenital adrenal hyperplasia","author":"Martinez","year":"2007","journal-title":"J Clin Endocrinol Metab"},{"key":"ref521","first-page":"9","article-title":"disgerminoma gonadoblastoma Ovarian neoplasms in children and adolescents","author":"Motlik","year":"1973"},{"key":"ref271","first-page":"16","article-title":"Endogenous androgens and risk of epithelial ovarian cancer : results from the European Prospective Investigation into Cancer EPIC","volume":"23","author":"Rinaldi","year":"2007","journal-title":"Nutrition Cancer Epidemiol Biomarkers"},{"key":"ref631","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1016\/j.ejca.2006.11.010","article-title":"the risk of epithelial ovarian cancer : a systematic meta - analysis","author":"Olsen","year":"2007","journal-title":"Obesity review Eur J Cancer"},{"key":"ref381","first-page":"521","article-title":"Ovarian masses in children and adolescents an analysis of clinical cases","volume":"27","author":"Zhang","year":"2014","journal-title":"J Pediatr Gynecol"},{"key":"ref71","doi-asserted-by":"crossref","DOI":"10.2169\/internalmedicine.50.5401","article-title":"woman with salt - wasting congenital adrenal hyperplasia presenting with a mucinous ovarian cystadenoma during pregnancy","author":"Kamata","year":"2011","journal-title":"Intern Med"},{"key":"ref551","first-page":"1975","article-title":"SEER cancer In editors Bethesda MD National","author":"Reis LA","year":"2004","journal-title":"statistics review Cancer Institute"},{"key":"ref241","doi-asserted-by":"crossref","DOI":"10.1093\/jnci\/90.23.1774","article-title":"Hormonal etiology of epithelial ovarian cancer with a hypothesis concerning the role of androgens and progesterone","author":"Risch HA","year":"1998","journal-title":"Natl Cancer Inst"},{"key":"ref561","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/S0090-8258(03)00407-4","article-title":"s syndrome due to ovarian serous adenocarcinoma secreting multiple endocrine substances : a case report and immunohistochemical analysis","author":"Suzuki","year":"2003","journal-title":"Gynecol Oncol"},{"key":"ref141","article-title":"NM AR HM Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia : results of ultrasonography and MR imaging","volume":"14","author":"Stikkelbroeck","year":"2004"},{"key":"ref261","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1002\/ijc.10990","article-title":"Circulating levels of sex steroid hormones and risk of ovarian cancer","author":"Lukanova","year":"2003","journal-title":"Int J Cancer"},{"key":"ref31","first-page":"37","article-title":"High frequency of nonclassical steroid hydroxylase deficiency","volume":"21","author":"Speiser","year":"1985","journal-title":"Am J Genet"},{"key":"ref351","first-page":"1","article-title":"New - phenotype correlation in families with congenital adrenal hyperplasia owing to - hydroxylase deficiency","author":"Abraham","year":"2013","journal-title":"Proc Natl Acad Sci USA"},{"key":"ref221","first-page":"1975","article-title":"SEER cancer In editors Bethesda MD National","author":"Reis LA","year":"2004","journal-title":"statistics review Cancer Institute"},{"key":"ref571","doi-asserted-by":"crossref","DOI":"10.1093\/jnci\/90.23.1774","article-title":"Hormonal etiology of epithelial ovarian cancer with a hypothesis concerning the role of androgens and progesterone","author":"Risch HA","year":"1998","journal-title":"Natl Cancer Inst"},{"key":"ref61","doi-asserted-by":"crossref","first-page":"549","DOI":"10.3109\/09513590.2014.907260","article-title":"Woman with virilizing congenital adrenal hyperplasia and leydig cell tumor of the ovary","volume":"30","author":"Fern\u00e1ndez","year":"2014","journal-title":"Gynecol Endocrinol"},{"key":"ref651","first-page":"5","article-title":"Ovarian serous carcinoma : recent concepts on its origin and","author":"Li","year":"2012","journal-title":"carcinogenesis Hematol Oncol"},{"key":"ref541","first-page":"95","article-title":"Congenital adrenal hyperplasia due to steroid hydroxylase deficiency an clinical practice guideline","volume":"21","author":"Speiser","year":"2010","journal-title":"Endocrine Society J Clin Endocrinol Metab"},{"key":"ref91","first-page":"706","article-title":"mutation frequencies and cancer penetrances : a kin - cohort study in Ontario","author":"Risch HA","year":"2006","journal-title":"Population Natl Cancer Inst"},{"key":"ref81","first-page":"125","article-title":"CA the past the future Markers","volume":"13","author":"Bast","year":"1998","journal-title":"Int J Biol"},{"key":"ref341","first-page":"866","article-title":"Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to hydroxylase deficiency","volume":"21","author":"Pang","year":"1988","journal-title":"Pediatrics"},{"key":"ref511","doi-asserted-by":"crossref","first-page":"69","DOI":"10.4158\/EP13092.CR","article-title":"Bilateral ovary adrenal rest tumor in a congenital adrenal hyperplasia following adrenalectomy","volume":"20","author":"Zaarour","year":"2014","journal-title":"Endocr Pract"},{"key":"ref581","first-page":"274","article-title":"Serum gonadotropins and steroid hormones and the development of ovarian cancer","volume":"30","author":"Helzlsouer","year":"1995","journal-title":"J Am Med Assoc"},{"key":"ref161","first-page":"109","article-title":"Bilateral Leydig cell tumor of the ovary in a woman with congenital adrenal hyperplasia The first reported case","volume":"20","author":"Luton","year":"1991","journal-title":"Presse Med"}],"container-title":["Journal of Pediatric Endocrinology and Metabolism"],"original-title":[],"link":[{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/jpem-2014-0299\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,22]],"date-time":"2021-04-22T01:37:59Z","timestamp":1619055479000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/jpem-2014-0299\/html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,1,1]]},"references-count":56,"journal-issue":{"issue":"5-6"},"URL":"https:\/\/doi.org\/10.1515\/jpem-2014-0299","relation":{},"ISSN":["2191-0251","0334-018X"],"issn-type":[{"value":"2191-0251","type":"electronic"},{"value":"0334-018X","type":"print"}],"subject":[],"published":{"date-parts":[[2015,1,1]]}}}