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Because of frequent disorder of genital development and a higher risk of germ cell neoplasia, management of these patients is complex and requires multidisciplinary approach.Case:\u00a0We present a 45,X\/46,XY mixed gonadal dysgenesis patient diagnosed with gonadoblastoma in both gonads after bilateral gonadectomy at 1 year of age.Conclusions:\u00a0Because of high risk for malignant transformation, gonadectomy of a streak-like gonad and biopsy with orchidopexy or gonadectomy of a dysgenetic testicle is recommended at an early age.<\/jats:p>","DOI":"10.15388\/amed.2022.29.2.5","type":"journal-article","created":{"date-parts":[[2022,7,25]],"date-time":"2022-07-25T03:52:22Z","timestamp":1658721142000},"page":"318-324","source":"Crossref","is-referenced-by-count":1,"title":["Early Bilateral Gonadoblastoma in a Patient with Mixed Gonadal Dysgenesis (Karyotype 45,X\/46,XY): Case Report and Review of Literature"],"prefix":"10.15388","volume":"29","author":[{"given":"Ignas","family":"Trainavi\u010dius","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darius","family":"Dasevi\u010dius","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5845-1045","authenticated-orcid":false,"given":"Birut\u0117","family":"Burnyt\u0117","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robertas","family":"Keme\u017eys","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7152-0802","authenticated-orcid":false,"given":"Gilvydas","family":"Verkauskas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"6097","published-online":{"date-parts":[[2022,7,25]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","unstructured":"ee PA, Nordenstr\u00f6m A, Houk CP, et al. 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