Objective: To analyze the clinical characteristics, treatments, and prognosis of patients with ovarian juvenile granulosa cell tumor (JGCT). Methods: Clinical and pathological data, and follow-up information of 34 patients diagnosed with JGCT from 2000 to 2021 were collected from the surveillance, epidemiology, and end results (SEER) database. A retrospective analysis was conducted to summarize the patients' clinical and pathological characteristics, treatments, and prognosis. Propensity score matching (PSM) was used to match the JGCT cases with adult granulosa cell tumor (AGCT) cases in SEER database. A total of 96 patients with ovarian granulosa cell tumor (OGCT), including 32 cases of JGCT and 64 cases of AGCT, were enrolled in a matched cohort analysis. Univariate and multivariate Cox regression analysis were performed on the matched cohort to explore the risk factors for overall survival. Kaplan-Meier curves and the log-rank test were used to compare the survival outcomes between JGCT and AGCT. Results: (1) The median age at diagnosis for the 34 JGCT patients was 19.5 years (ranged: 1-48 years), with 3 patients aged ≤10 years, 16 patients aged 11-20 years, 11 patients aged 21-30 years, and 4 patients aged >30 years. Tumors originated unilaterally in 33 patients, with only 1 case originating bilaterally. The maximum tumor diameter was recorded in 26 patients, with a median size of 12.4 cm (ranged: 3.5-40.0 cm). According to the 2014 International Federation of Gynecology and Obstetrics (FIGO) staging system, 19 patients were diagnosed with stage Ⅰ (including 10 cases with stage Ⅰa and 9 cases with stage Ⅰc), 4 patients with stage Ⅱ, 8 patients with stage Ⅲ, and 3 patients with stage Ⅳ. Two patients did not undergo surgery for the resection of lesions. Stage Ⅰ patients (15/19) underwent fertility-sparing surgery, while stage Ⅱ-Ⅲ patients underwent either fertility-sparing surgery or cytoreductive surgery (6 cases each). Stage Ⅳ patients underwent cytoreductive surgery (2 cases). Lymph node dissection was performed in 10 patients, among which only 1 patient with positive lymph nodes metastasis. None of the 34 patients received radiotherapy, while 18 patients received adjuvant chemotherapy (included neoadjuvant chemotherapy and postoperative adjuvant chemotherapy). The proportion of stage Ⅰ patients receiving adjuvant chemotherapy was relatively low, with only 4 out of 19 patients (including 2 out of 10 cases for stage Ⅰa and 2 out of 9 cases for stage Ⅰc). The proportions of patients receiving adjuvant chemotherapy for stages Ⅱ, Ⅲ and Ⅳ were 3 out of 4 cases, 8 out of 8 cases, and 3 out of 3 cases, respectively. The follow-up ended in December 2021, with 20 patients alive and 14 dead. The survival rate for ovarian JGCT patients was 59% (20/34). Among them, the survival rate for stage Ⅰ patients was 16/19, while for stage Ⅱ-Ⅳ patients, it was 4/15; there was a statistically significant difference (P=0.002). Among stage Ⅱ-Ⅲ patients, the survival rate at the end of follow-up was 1/6 for those who underwent fertility-sparing surgery, compared to 3/6 for those who underwent cytoreductive surgery (P=0.546). (2) For the 96 OGCT patients after matching using the PSM method, 64 ovarian AGCT patients had 5 deaths and 59 survivors during the follow-up period, the survival rate was 92% (59/64) at the end of follow-up. In contrast, among the 32 ovarian JGCT patients, 13 died and 19 survived, resulting in a survival rate of 59% (19/32) at the end of follow-up, which was statistically significant difference for the AGCT group (P<0.001). Univariate Cox analysis revealed that histology, extent of surgery, chemotherapy, postoperative tumor residual status, and stage all significantly affected the survival outcomes of OGCT patients (all P<0.05). Multivariate Cox analysis revealed that variables with significant statistical differences were histology and stage. The median survival time for JGCT patients was 126 months, while AGCT patients median survival time was not reached with a statistically significant between the two groups (P<0.001). Conclusions: Ovarian JGCT predominantly occur in adolescents and young women. Lymph node metastasis is relatively rare, and treatment primarily involves surgery and adjuvant chemotherapy. Most ovarian JGCT patients are diagnosed at stage Ⅰ, with a favorable prognosis. Fertility-preserving surgery is recommended, involving salpingo-oophorectomy on the affected side plus comprehensive staging surgery, or a second surgery to achieve comprehensive staging. For stage Ⅱ-Ⅳ ovarian JGCT patients, the prognosis is relatively poor, and fertility-preserving surgery should be considered with caution. The prognosis of ovarian JGCT patients is worse than that of ovarian AGCT patients.
目的： 探讨卵巢幼年型颗粒细胞瘤（JGCT）患者的临床病理特征、治疗及预后。 方法： 从美国国立癌症研究所的监测、流行病学及最终结果（SEER）数据库中收集2000—2021年期间诊断为卵巢JGCT的34例患者的临床病理资料及随访资料进行回顾性分析，总结卵巢JGCT患者的临床病理特征、治疗及预后。采用倾向性评分匹配（PSM）方法匹配SEER数据库中的卵巢成人型颗粒细胞瘤（AGCT）64例，共有96例卵巢颗粒细胞瘤（OGCT）患者（包括32例JGCT和64例AGCT）纳入匹配后的队列分析，对匹配后队列采用单因素及多因素Cox回归模型分析其预后影响因素；并采用Kaplan-Meier法绘制生存曲线，log-rank检验比较JGCT与AGCT患者的预后差异。 结果： （1）34例卵巢JGCT患者的中位诊断年龄为19.5岁（范围：1~48岁），其中≤10岁3例，11~20岁16例，21~30岁11例，>30岁4例；肿瘤位置：33例患者的肿瘤位于单侧卵巢，仅1例Ⅳ期患者的肿瘤位于双侧卵巢；肿瘤最大径：26例患者有记录，中位肿瘤最大径为12.4 cm（范围：3.5~40.0 cm）；国际妇产科联盟（FIGO）2014年分期：Ⅰ期19例（其中Ⅰa期10例、Ⅰc期9例），Ⅱ期4例，Ⅲ期8例，Ⅳ期3例。手术方式：有2例患者（Ⅰc、Ⅳ期各1例）未对病灶行手术治疗，多数Ⅰ期患者（15/19）行保留生育功能手术，Ⅱ~Ⅲ期患者行保留生育功能手术或肿瘤细胞减灭术（各6例），Ⅳ期患者（2例）行肿瘤细胞减灭术；淋巴结转移：术中切除淋巴结的患者共有10例，其中仅1例患者淋巴结阳性；放化疗：34例患者术后均未接受放疗，18例患者接受化疗（包括术前和术后化疗），其中Ⅰ期患者接受的化疗比例较低，仅为4/19（其中Ⅰa期2/10、Ⅰc期2/9），Ⅱ、Ⅲ、Ⅳ期患者分别为3/4、8/8、3/3。随访截止时间为2021年12月，随访期内20例患者存活、14例死亡，卵巢JGCT患者的总生存（OS）率为59%（20/34）；其中，Ⅰ期患者为16/19，Ⅱ~Ⅳ期患者为4/15，两者比较，差异有统计学意义（P=0.002）；Ⅱ~Ⅲ期患者中，保留生育功能手术患者的OS率为1/6，低于肿瘤细胞减灭术患者的3/6，但两者比较，差异无统计学意义（P=0.546）。（2）PSM方法匹配后的96例OGCT患者中，64例卵巢AGCT患者随访期内5例死亡、59例存活，卵巢AGCT患者的OS率为92%（59/64），而32例卵巢JGCT患者中死亡13例、存活19例，卵巢JGCT患者的OS率为59%（19/32），两者比较，差异有统计学意义（P<0.001）。单因素分析显示，病理类型、手术方式、化疗、术后残留灶、FIGO分期均显著影响OGCT患者的预后（P均<0.05）；多因素分析显示，病理类型为JGCT、FIGO分期为Ⅲ~Ⅳ期均是影响OGCT患者预后的独立危险因素（P均<0.05）。JGCT患者的中位OS时间为126个月，而AGCT患者的中位OS时间随访期内未达到，两者比较，差异有统计学意义（P<0.001）。 结论： 卵巢JGCT多发生于少年儿童及年轻女性，淋巴结转移相对较少，治疗以手术及化疗为主。大多数卵巢JGCT患者诊断时为Ⅰ期，预后较好，可行保留生育功能手术，手术方式推荐患侧附件切除+全面分期手术或者二次手术达到全面分期的目的；Ⅱ~Ⅳ期卵巢JGCT患者的预后相对较差，应谨慎考虑行保留生育功能手术。卵巢JGCT患者的预后劣于卵巢AGCT患者。.