卵巢幼年型颗粒细胞瘤：来自国际卵巢和睾丸间质瘤及国际胸膜肺母细胞瘤/DICER1登记处的报告。
Ovarian juvenile granulosa cell tumor: A report from the International Ovarian and Testicular Stromal Tumor and International Pleuropulmonary Blastoma/DICER1 Registries.
作者信息
Harris Anne K, Nelson Alexander T, Watson Dave, Mallinger Paige H R, Messinger Yoav H, Frazier A Lindsay, Stering Allen, Snyder Stacy L, Levy Carolyn Fein, Kamihara Junne, Herzog Cynthia E, Lagmay Joanne, Foresto Steve, Chen Kenneth S, Devins Kyle M, Young Robert H, Hill D Ashley, Dehner Louis P, Tadavarthy Anil K, Stall Jennifer N, Billmire Deborah F, Schneider Dominik T, Schultz Kris Ann P
机构信息
International Pleuropulmonary Blastoma/DICER1 Registry, Children's Minnesota, Minneapolis, Minnesota, USA.
International Ovarian and Testicular Stromal Tumor Registry, Children's Minnesota, Minneapolis, Minnesota, USA.
出版信息
Cancer. 2025 May 1;131(9):e35862. doi: 10.1002/cncr.35862.
BACKGROUND
Ovarian juvenile granulosa cell tumors (juvGCT) are rare sex cord-stromal tumors that occur primarily in children and adolescents. This study summarizes the clinical presentation and outcomes of patients with juvGCT.
METHODS
Patients were enrolled in the International Ovarian and Testicular Stromal Tumor and/or International Pleuropulmonary Blastoma/DICER1 Registries. Available medical records were abstracted, and pathology was centrally reviewed. Surgical staging was classified using the 2014 International Federation of Gynecology and Obstetrics (FIGO) criteria.
RESULTS
In total, 70 patients with juvGCT enrolled and were diagnosed between 2001 and 2024; most patients (81%, 57 of 70) presented with FIGO stage I disease. Adjuvant chemotherapy was given in 30% (21 of 70); all regimens were platinum-based. Three-year event-free survival among patients with stage IA tumors was 80.2% (95% confidence interval [CI], 62.4%-100.0%), IC1 was 87.4 (95% CI, 72.4%-100.0%), IC2-IC3 was 63.6% (95% CI, 40.7%-99.5%), and II-IV was 48% (95% CI, 24.6%-93.8%). Of the patients with recurrent juvGCT with known mitotic index (MI), all had MI greater than 19 mitoses per 10 high power fields (HPF) at diagnosis.
CONCLUSION
Outcomes were worse for patients with FIGO stage ≥IC2 disease and for tumors with >19 mitoses per 10 HPF. Given the prognostic significance of MI, the authors strongly recommend the assessment of MI for all juvGCTs. More information about tumor biology is critical to identify which patients may benefit from adjuvant chemotherapy and to facilitate the development of novel therapies.
背景
卵巢幼年型颗粒细胞瘤（juvGCT）是一种罕见的性索间质肿瘤，主要发生于儿童和青少年。本研究总结了juvGCT患者的临床表现及治疗结果。
方法
患者被纳入国际卵巢和睾丸间质肿瘤及/或国际胸膜肺母细胞瘤/DICER1登记处。提取可用的病历，并进行集中病理审查。手术分期采用2014年国际妇产科联盟（FIGO）标准进行分类。
结果
2001年至2024年期间，共有70例juvGCT患者入组并被诊断；大多数患者（81%，70例中的57例）表现为FIGO I期疾病。30%（70例中的21例）接受了辅助化疗；所有方案均以铂类为基础。IA期肿瘤患者的3年无事件生存率为80.2%（95%置信区间[CI]，62.4%-100.0%），IC1期为87.4%（95%CI，72.4%-100.0%），IC2-IC3期为63.6%（95%CI，40.7%-99.5%），II-IV期为48%（95%CI，24.6%-93.8%）。在已知有丝分裂指数（MI）的复发性juvGCT患者中，所有患者在诊断时的MI均大于每10个高倍视野（HPF）19个有丝分裂。
结论
FIGO分期≥IC2期疾病的患者以及每10个HPF有丝分裂>19个的肿瘤患者的预后较差。鉴于MI的预后意义，作者强烈建议对所有juvGCT进行MI评估。更多关于肿瘤生物学的信息对于确定哪些患者可能从辅助化疗中获益以及促进新疗法的开发至关重要。
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