{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T07:08:04Z","timestamp":1756192084360},"reference-count":80,"publisher":"Walter de Gruyter GmbH","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,5,27]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"j_jpem-2018-0512_s_999\"><jats:title id=\"d2783957e35095\">Background<\/jats:title><jats:p>Testicular adrenal rest tumors (TARTs) leading to primary gonadal failure are the main etiology of infertility in congenital adrenal hyperplasia (CAH). We aimed at identifying the evolution of TART and related findings in young CAH patients.<\/jats:p><\/jats:sec><jats:sec id=\"j_jpem-2018-0512_s_998\"><jats:title id=\"d2783957e35103\">Methods<\/jats:title><jats:p>Twelve male patients (3\u201323 years old) with 21-hydroxilase deficiency (11 with classic salt-wasting form) were included. Testicular ultrasonography (US) was performed in two moments, by a single blinded specialist in pediatric diagnostic imaging. Tumor progression was classified according to the Response Evaluation Criteria in Solid Tumors (RECIST). The clinical and laboratory data were retrieved from medical records. Serum 17-OH-progesterone (17OHP) and androstenedione concentrations were evaluated during the whole period of follow-up, from the CAH diagnosis. A logistic regression model with repeated measures was developed for the analysis.<\/jats:p><\/jats:sec><jats:sec id=\"j_jpem-2018-0512_s_997\"><jats:title id=\"d2783957e35111\">Results<\/jats:title><jats:p>The prevalence of TART was 41.6% (n\u2009=\u20095) in the initial US evaluation and 66.6% (n\u2009=\u20098) after 6 years of follow-up. Tumor progression was detected in 4 of the 5 patients, and 1 presented with a stable tumor. Three patients presented with new tumors in the second evaluation. Most of the patients (n\u2009=\u200911) were pubertal, including a 7-year-old child with TART who presented with central precocious puberty. At regression analysis, it was observed that an inadequate hormonal control led to a 16 times greater chance of a patient to present with TART (OR\u2009=\u200916.08; confidence interval [CI] 95%\u2009=\u20092.38\u2013108.81; p\u2009=\u20090.004).<\/jats:p><\/jats:sec><jats:sec id=\"j_jpem-2018-0512_s_996\"><jats:title id=\"d2783957e35119\">Conclusions<\/jats:title><jats:p>We found a high prevalence of progressive TART in young pubertal subjects. US testicular screening should help in improving therapeutic optimization in CAH patients to reduce future impairment in fertility.<\/jats:p><\/jats:sec>","DOI":"10.1515\/jpem-2018-0512","type":"journal-article","created":{"date-parts":[[2019,5,10]],"date-time":"2019-05-10T09:02:41Z","timestamp":1557478961000},"page":"519-526","source":"Crossref","is-referenced-by-count":6,"title":["Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia: 6 years of follow-up"],"prefix":"10.1515","volume":"32","author":[{"given":"Gabriela","family":"Werneck","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enda M.R.","family":"Rodrigues","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rafael M.","family":"Mantovani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jovita S.S.","family":"Lane","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ivani N.","family":"Silva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref361","doi-asserted-by":"crossref","first-page":"155.e1","DOI":"10.1016\/j.jpurol.2017.11.011","article-title":"Testicular adrenal rest tumor screening and fertility counseling among males with congenital adrenal hyperplasia","volume":"14","year":"2018","journal-title":"J Pediatr Urol"},{"key":"ref531","doi-asserted-by":"crossref","first-page":"5721","DOI":"10.1210\/jcem.86.12.8090","article-title":"High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia","volume":"86","year":"2001","journal-title":"J Clin Endocrinol 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