Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma (RPS): A Report on 1007 Patients From the Multi-institutional Collaborative RPS Working Group

Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma (RPS): A Report on 1007 Patients From the Multi-institutional Collaborative RPS Working Group
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DOI:
10.1097/sla.0000000000001447
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发表时间:
2016-05-01
期刊:
影响因子:
9
通讯作者:
Raut, Chandrajit P.
Raut, Chandrajit P.
中科院分区:
医学1区
文献类型:
--
作者:
Gronchi, Alessandro;Strauss, Dirk C.;Raut, Chandrajit P.

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背景:腹膜后肉瘤(RPS)是一种罕见的肿瘤,由几种明确的组织学亚型组成。本研究的目的是分析模式的复发和治疗的变化,在一个大的人口的患者,治疗在reference centers.Methods:2002年1月和2011年12月之间,所有连续的患者与主要RPS在6个欧洲和2个北美机构治疗。计算5年、8年和10年的总生存率(OS)以及局部复发(LR)和远处转移(DM)的粗累积发生率(CCI)。对OS、LR的CCI和DM进行多因素分析。结果:共纳入1007例患者。中位随访时间为58个月(第一和第三四分位数范围36-90)。5、8和10年OS分别为67% [95%置信区间(CI),63,70)、56%(95% CI,52,61)和46%(95% CI,40,53)。LR和DM的5、8和10年CCI分别为25.9(95% CI,23.1,29.1),31.3(95% CI,27.8,35.1)、35%(95% CI,30.5,40.1)和21%(95% CI,18.4,23.8%)、21.6(95% CI,19.0,24.6)和21.6(95% CI,19.0,24.6)。肿瘤大小、组织学亚型、恶性程度、多灶性和切除完整性是预后的重要预测因素。复发模式根据组织学亚型而不同。不同的治疗政策在参与机构的影响LR分化良好的脂肪肉瘤不影响OS,而辅助系统治疗的差异并没有影响LR,DM,或OS的平滑肌肉瘤。结论:参考中心是至关重要的RPS患者的结果,作为管理策略需要具体的专业知识。组织学亚型可预测复发模式,并应告知管理决策。目前正在筹备一项前瞻性的国际登记,以进一步确定我们对这一疾病的认识。
Background:Retroperitoneal sarcomas (RPS) are rare tumors composed of several well defined histologic subtypes. The aim of this study was to analyze patterns of recurrence and treatment variations in a large population of patients, treated at reference centers.Methods:All consecutive patients with primary RPS treated at 6 European and 2 North American institutions between January 2002 and December 2011 were included. Five, 8, and 10-year overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were calculated. Multivariate analyses for OS, CCI of LR, and DM were performed.Results:In all, 1007 patients were included. Median follow-up was 58 months (first and third quartile range 36-90). The 5, 8, and 10-year OS were 67% [95% confidence interval (CI), 63, 70), 56% (95% CI, 52, 61), and 46% (95% CI, 40, 53). The 5, 8, and 10-year CCI of LR and DM were 25.9 (95% CI, 23.1, 29.1), 31.3 (95% CI, 27.8, 35.1), 35% (95% CI, 30.5, 40.1), and 21% (95% CI, 18.4, 23.8%), 21.6 (95% CI, 19.0, 24.6), and 21.6 (95% CI, 19.0, 24.6), respectively. Tumour size, histologic subtype, malignancy grade, multifocality, and completeness of resection were significant predictors of outcome. Patterns of recurrence varied depending on histologic subtype. Different treatment policies at participating institutions influenced LR of well differentiated liposarcoma without impacting OS, whereas discrepancies in adjuvant systemic therapies did not impact LR, DM, or OS of leiomyosarcoma.Conclusions:Reference centers are critical to outcomes of RPS patients, as the management strategy requires specific expertise. Histologic subtype predicts patterns of recurrence and should inform management decision. A prospective international registry is under preparation, to further define our understanding of this disease.