Department of Urology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Department of Endocrinology, Genetics and Metabolism, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Asian J Androl. 2024 Nov 1;26(6):617-621. doi: 10.4103/aja202445. Epub 2024 Aug 9.
Central precocious puberty secondary to Leydig cell tumors is rare in children. We retrospectively analyzed the mid- to long-term follow-up data of patients with Leydig cell tumors. The clinical data of 12 consecutive patients who were treated at Beijing Children's Hospital, Capital Medical University (Beijing, China), between January 2016 and October 2023 were retrospectively reviewed. Clinical evaluations, including physical examination, hormone examination, serum tumor marker analysis, abdominal and scrotal ultrasound, chest X-ray, and bone age measurement, were conducted before surgery and at follow-up time points. Surgical approaches were selected according to the individual conditions. Patients with an abnormal hormonal status and suspected of having central precocious puberty were referred to endocrinologists to confirm the diagnosis. Subsequently, gonadotropin-releasing hormone analog therapy was proposed. The mean patient age was 81.3 (range: 40-140) months at the time of the operation. Ten patients had peripheral precocious puberty at admission. All patients had elevated preoperative testosterone levels, whereas tumor marker levels were normal. Testis-sparing surgery was performed in eleven patients, and radical orchiectomy was performed in one patient. The follow-up duration (mean ± standard deviation) was 36.2 ± 25.3 months. Five patients had central precocious puberty, with a mean duration of 3.4 (range: 1-6) months postoperatively. Three patients were receiving gonadotropin-releasing hormone analog therapy, and good suppression of puberty was observed. No risk factors were found for secondary central precocious puberty. There was a high prevalence of central precocious puberty secondary to Leydig cell tumors in our study. Gonadotropin-releasing hormone analog therapy has satisfactory treatment effects. Larger sample sizes and long-term follow-up are needed in future studies.
儿童促性腺激素释放激素细胞腺瘤继发中枢性性早熟罕见。我们回顾性分析了促性腺激素释放激素细胞腺瘤患者的中远期随访资料。回顾性分析 2016 年 1 月至 2023 年 10 月首都医科大学附属北京儿童医院收治的 12 例连续患者的临床资料，所有患者均经手术治疗。术前及随访时进行临床评估，包括体格检查、激素检查、血清肿瘤标志物分析、腹部及阴囊超声、胸部 X 线片及骨龄测定。根据个体情况选择手术方式。对存在激素水平异常、疑诊中枢性性早熟的患者，转至内分泌科进行确诊，随后给予促性腺激素释放激素类似物治疗。患者的平均手术年龄为 81.3（40140）月龄，入院时 10 例患者存在外周性性早熟，所有患者术前睾酮水平升高，肿瘤标志物水平正常。11 例行保留睾丸的手术，1 例行根治性睾丸切除术。随访时间（均数±标准差）为 36.2±25.3 个月。5 例患者发生中枢性性早熟，术后平均持续 3.4（16）个月。3 例患者接受促性腺激素释放激素类似物治疗，性发育抑制良好。未发现继发中枢性性早熟的危险因素。本研究中促性腺激素释放激素细胞腺瘤继发中枢性性早熟的发生率较高，促性腺激素释放激素类似物治疗效果满意，需要进一步开展大样本量及长期随访的研究。
