{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T23:01:11Z","timestamp":1772578871532,"version":"3.50.1"},"reference-count":34,"publisher":"The Endocrine Society","issue":"11","license":[{"start":{"date-parts":[[2022,9,8]],"date-time":"2022-09-08T00:00:00Z","timestamp":1662595200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,23]]},"abstract":"Abstract<\/jats:title>\n \n Objective<\/jats:title>\n The purpose of this study is to report development of a malignant testicular germ cell tumor (GCT) in 2 young adult males with familial male-limited precocious puberty (FMPP) because of LHCGR pathogenic variants in 2 families. Secondarily, to study the possible relation between FMPP and testicular tumors and to investigate whether FMPP might predispose to development of malignant testicular tumors in adulthood a literature review is conducted.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n Data on 6 cases in 2 families are obtained from the available medical records. In addition, a database search is performed in Cochrane, PubMed, and Embase for studies that report on a possible link between FMPP and testicular tumors.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n The characteristics of 6 males with FMPP based on activating LH receptor (LHCGR) germline pathogenic variants are described, as are details of the testicular GCTs. Furthermore, a literature review identified 4 more patients with signs of FMPP and a (precursor of) testicular GCT in adolescence or adulthood (age 15-35 years). Additionally, 12 patients with signs of precocious puberty and, simultaneously, occurrence of a Leydig cell adenoma or Leydig cell hyperplasia are reported.<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n There is a strong suggestion that FMPP might increase the risk of development of testicular GCTs in early adulthood compared with the risk in the general population. Therefore, prolonged patient monitoring from mid-pubertal age onward including instruction for self-examination and periodic testicular ultrasound investigation in patients with a germline LHCGR pathogenic variant might contribute to early detection and thus early treatment of testicular GCT.<\/jats:p>\n <\/jats:sec>","DOI":"10.1210\/clinem\/dgac516","type":"journal-article","created":{"date-parts":[[2022,9,8]],"date-time":"2022-09-08T03:48:31Z","timestamp":1662608911000},"page":"3035-3044","source":"Crossref","is-referenced-by-count":7,"title":["Familial Male-limited Precocious Puberty (FMPP) and Testicular Germ Cell Tumors"],"prefix":"10.1210","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5985-0075","authenticated-orcid":false,"given":"Cezanne D","family":"Kooij","sequence":"first","affiliation":[{"name":"Princess Ma\u0301xima Center for Pediatric Oncology , 3584 CS Utrecht , The 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