Li Zixiang, Liu Junjie, Peng Yunpeng, Chen Renfu, Ge Peng, Wang Junqi
Department of Urology, the Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu, China.
Medicine (Baltimore). 2020 Oct 2;99(40):e22530. doi: 10.1097/MD.0000000000022530.
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.
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.
46, XX ovotesticular DSD with seminoma.
The patient was treated with neochemotherapy (etoposide and cisplatin), surgery, chemotherapy, and testosterone replacement.
At the 13-month follow-up, the patient reported satisfactory erections, and no evidence of disease was found.
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.
性发育障碍（DSD）中的卵睾性DSD，以前称为真两性畸形，是一种个体同时具有睾丸和卵巢组织的疾病。46,XX卵睾性DSD个体性腺中出现肿瘤的情况并不常见。
我们报告一例36岁的表型男性患者，主诉腹部肿块3个月。他自述勃起正常且月经规律。计算机断层扫描显示一个大小为15×10厘米的大肿瘤、一个子宫和一个囊性卵巢。
46,XX卵睾性DSD伴精原细胞瘤。
患者接受了新辅助化疗（依托泊苷和顺铂）、手术、化疗及睾酮替代治疗。
在13个月的随访中，患者报告勃起情况令人满意，且未发现疾病迹象。
46,XX卵睾性DSD伴精原细胞瘤的病例并不常见。我们的病例揭示了手术联合新辅助化疗、化疗及睾酮替代治疗对改善这些患者预后的重要性。
