{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T20:22:34Z","timestamp":1785270154604,"version":"3.55.0"},"reference-count":17,"publisher":"S. Karger AG","issue":"3","license":[{"start":{"date-parts":[[2024,4,16]],"date-time":"2024-04-16T00:00:00Z","timestamp":1713225600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2024,4,16]],"date-time":"2024-04-16T00:00:00Z","timestamp":1713225600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"abstract":"<jats:p>&lt;p&gt;Introduction: Ovarian Sertoli cell tumors represent a subset of sex cord stromal tumors and are exceedingly rare in prepubertal children. Here, we report a girl with vaginal bleeding due to a Sertoli cell tumor who was originally thought to have McCune-Albright syndrome (MAS). Case Presentation: A previously healthy girl presented at age 2 years 6 months with breast development and vaginal bleeding. On exam, she had Tanner 4 breasts, Tanner 1 pubic hair, estrogenized vaginal mucosa, and a caf\u00e9-au-lait macule. Laboratory studies revealed an elevated estradiol with suppressed gonadotropins and negative tumor markers. Her bone age was advanced by more than 3 years. Pelvic ultrasound (US) revealed an enlarged uterus and a slightly larger left compared to right ovary. She was started on tamoxifen for presumed MAS. A repeat pelvic US 1 month later showed a heterogenous mass in the left ovary which was subsequently resected. Pathology revealed a Sertoli cell tumor, lipid-rich variant. Germline sequencing revealed a pathogenic STK11 variant, diagnostic for Peutz-Jeghers syndrome (PJS). Conclusion: The findings in our patient were strikingly similar to those encountered in MAS. To our knowledge, our patient is the youngest ever reported to present with precocious puberty due to a Sertoli cell tumor in the setting of PJS. &lt;\/p&gt;<\/jats:p>","DOI":"10.1159\/000538945","type":"journal-article","created":{"date-parts":[[2024,4,16]],"date-time":"2024-04-16T17:00:31Z","timestamp":1713286831000},"page":"362-367","source":"Crossref","is-referenced-by-count":2,"title":["Diagnostic Conundrum of a Sertoli Cell Tumor in a 2-Year-Old Girl with Peripheral Precocious Puberty and a Caf\u00e9-au-Lait Macule: A Case Report"],"prefix":"10.1159","volume":"98","author":[{"given":"Lauren A.","family":"Ray","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah F.","family":"Billmire","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J.","family":"Ferguson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6135-7474","authenticated-orcid":false,"given":"Erica A.","family":"Eugster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2024,4,16]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-3-12"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2020.00522"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1159\/000504802"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2007.04.021"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(03)00128-8"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1148\/rg.347130144"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198609000-00010"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2014.184"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1016\/0030-4220(57)90116-0"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1016\/s0007-117x(68)80034-4"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000149692.21205.9c"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1097\/MOG.0000000000000718"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1159\/000529799"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1038\/ajg.2014.435"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2022.02.004"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-006-0352-4"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1006\/gyno.1998.5063"}],"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/hrp\/article-pdf\/doi\/10.1159\/000538945\/4231970\/000538945.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000538945","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,19]],"date-time":"2025-09-19T16:03:59Z","timestamp":1758297839000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000538945"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,16]]},"references-count":17,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,6,3]]},"published-print":{"date-parts":[[2025,6,2]]}},"URL":"https:\/\/doi.org\/10.1159\/000538945","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,16]]}}}