Abstract：
BACKGROUND: Malignant sex cord-stromal cell tumours (SCST) account for only 7% of ovarian malignancies. The Arbeitsgemeinschaft fuer Gynaekologische Onkologie (AGO) study group has established a clinicopathological database to provide an overview of the current treatment strategies and survival of SCST patients and to identify research needs.
METHODS: Twenty centres provided mixed retro- and prospective data of patients with tumour specimens and second-opinion pathology review treated between 2000 and 2014. Descriptive analyses of treatment strategies, Kaplan-Meier curves and cox regression analyses were conducted.
RESULTS: Two hundred and sixty-two SCST patients were included. One hundred and ninety-one Granulosa-cell tumour (GCT) and 17 Sertoli-Leydig cell tumour (SLCT) patients were stage I disease (>80%). Forty four GCT (18.7%) and two (8.3%) SLCT patients received adjuvant systemic treatment. After a median observation time of 78.2 months, 46% of all SCST patients experienced disease recurrence, treated predominantly with secondary debulking surgery (> 90%). Advanced FIGO stage, lymph node involvement and intra-operative capsule rupture were associated with disease recurrence on univariate analysis (all p < 0.05). Median OS time was not reached.
CONCLUSIONS: In this analysis of SCST patients, adjuvant chemotherapy was unable to prevent disease recurrence. Despite high recurrence rates, overall survival rates were excellent.
METHODS: Twenty centres provided mixed retro- and prospective data of patients with tumour specimens and second-opinion pathology review treated between 2000 and 2014. Descriptive analyses of treatment strategies, Kaplan-Meier curves and cox regression analyses were conducted.
RESULTS: Two hundred and sixty-two SCST patients were included. One hundred and ninety-one Granulosa-cell tumour (GCT) and 17 Sertoli-Leydig cell tumour (SLCT) patients were stage I disease (>80%). Forty four GCT (18.7%) and two (8.3%) SLCT patients received adjuvant systemic treatment. After a median observation time of 78.2 months, 46% of all SCST patients experienced disease recurrence, treated predominantly with secondary debulking surgery (> 90%). Advanced FIGO stage, lymph node involvement and intra-operative capsule rupture were associated with disease recurrence on univariate analysis (all p < 0.05). Median OS time was not reached.
CONCLUSIONS: In this analysis of SCST patients, adjuvant chemotherapy was unable to prevent disease recurrence. Despite high recurrence rates, overall survival rates were excellent.
摘要：
背景：恶性性索基质细胞瘤（SCST）仅占卵巢恶性肿瘤的7%。GynaekologischeOnkologie(AGO)研究小组已经建立了一个临床病理数据库，以提供SCST患者当前治疗策略和生存的概述，并确定研究需求。
方法：20个中心提供了2000年至2014年间接受治疗的肿瘤标本和第二意见病理学回顾患者的混合回顾性和前瞻性数据。治疗策略的描述性分析，进行Kaplan-Meier曲线和cox回归分析。
结果：纳入了二百六十二例SCST患者。101例颗粒细胞瘤（GCT）和17例Sertoli-Leydig细胞瘤（SLCT）患者为I期疾病（>80％）。44例GCT（18.7%）和2例SLCT（8.3%）患者接受了辅助全身治疗。在中位观察时间为78.2个月后，所有SCST患者中有46％经历了疾病复发，主要采用二次减瘤手术治疗（>90％）。高级FIGO阶段，单因素分析显示淋巴结受累及术中包膜破裂与疾病复发相关(均P<0.05)。未达到中位OS时间。
结论：在对SCST患者的分析中，辅助化疗不能预防疾病复发。尽管复发率高,总体生存率优异.
方法：20个中心提供了2000年至2014年间接受治疗的肿瘤标本和第二意见病理学回顾患者的混合回顾性和前瞻性数据。治疗策略的描述性分析，进行Kaplan-Meier曲线和cox回归分析。
结果：纳入了二百六十二例SCST患者。101例颗粒细胞瘤（GCT）和17例Sertoli-Leydig细胞瘤（SLCT）患者为I期疾病（>80％）。44例GCT（18.7%）和2例SLCT（8.3%）患者接受了辅助全身治疗。在中位观察时间为78.2个月后，所有SCST患者中有46％经历了疾病复发，主要采用二次减瘤手术治疗（>90％）。高级FIGO阶段，单因素分析显示淋巴结受累及术中包膜破裂与疾病复发相关(均P<0.05)。未达到中位OS时间。
结论：在对SCST患者的分析中，辅助化疗不能预防疾病复发。尽管复发率高,总体生存率优异.
