46, XX Ovotesticular disorder of sex development (true hermaphroditism) with seminoma: A case report.

Abstract

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.