Open Access46, XX Ovotesticular disorder of sex development (true hermaphroditism) with seminoma
Author(s) -
Zixiang Li,
Junjie Liu,
Yunpeng Peng,
Renfu Chen,
P. Ge,
Junqi Wang
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000022530
Subject(s) - medicine , true hermaphroditism , seminoma , etoposide , ovary , orchiopexy , testosterone (patch) , uterus , chemotherapy , surgery , gynecology , karyotype , biochemistry , chemistry , chromosome , gene
Rationale: Ovotesticular disorder of sex development (DSD), previously known as true hermaphroditism, is a disorder in which individuals have both testicular and ovarian tissues. Instances of tumors arising in the gonads of individuals with 46,XX ovotesticular DSD are uncommon. Patient concerns: We report a case of a 36-year-old phenotypical male with a chief complaint of an abdominal mass for 3 months. He reported normal erections and regular menses. Computerized tomography showed a large tumor measuring 15 × 10 cm in size, a uterus, and a cystic ovary. Diagnosis: 46, XX ovotesticular DSD with seminoma. Interventions: The patient was treated with neochemotherapy (etoposide and cisplatin), surgery, chemotherapy, and testosterone replacement. Outcomes: At the 13-month follow-up, the patient reported satisfactory erections, and no evidence of disease was found. Conclusion: Cases of 46,XX ovotesticular DSD with seminoma are uncommon. Our case reveals the importance of surgery combined with neochemotherapy, chemotherapy, and testosterone replacement in these patients to improve the prognosis.