title: Pure non-gestational ovarian choriocarcinoma in a 45,XO/46,XX SRY-negative true hermaphrodite.
abstract: Non-gestational ovarian choriocarcinoma (NGCO) is an extremely rare malignant tumor with a poor prognosis and is difficult to distinguish from gestational choriocarcinoma. True hermaphrodite (TH) is genetically a heterogenous condition causing ovarian and testicular tissue development in the same individual. We report here the first case of pure NGCO in the right ovotestis of a 23-year-old 45,XO/46X,X sex-determining region Y chromosome (SRY)-negative TH. The diagnosis of non-gestational origin was confirmed by testing five short tandem repeats (STR). The patient responded well to radical surgery with bleomycin, etoposide, cisplatin (BEP) regimen. We also hypothesize that some mutations of an X-linked or autosomal gene lead to testicular determination in SRY-negative TH patients.
authors: Department of Gynecology and Obstetrics, West China Second University Hospital, Sichuan University, Sichuan, China.
authors: Ting
authors: T
authors: Hu
authors: Mei
authors: M
authors: Yang
authors: Hongmei
authors: H
authors: Zhu
authors: Gang
authors: G
authors: Shi
authors: He
authors: H
authors: Wang
mesh_terms: D031954:Choriocarcinoma, Non-gestational / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000235:genetics / Q000473:pathology*; D050090:Ovotesticular Disorders of Sex Development / Q000235:genetics / Q000473:pathology*; D055815:Young Adult