PDF(Pubmed)
Abstract：
UNASSIGNED: This study aimed to assess the performance of currently available risk calculators in a cohort of patients with malignant peripheral nerve sheath tumors (MPNST) and to create an MPNST-specific prognostic model including type-specific predictors for overall survival (OS).
UNASSIGNED: This is a retrospective multicenter cohort study of patients with MPNST from 11 secondary or tertiary centers in The Netherlands, Italy and the United States of America. All patients diagnosed with primary MPNST who underwent macroscopically complete surgical resection from 2000 to 2019 were included in this study. A multivariable Cox proportional hazard model for OS was estimated with prespecified predictors (age, grade, size, NF-1 status, triton status, depth, tumor location, and surgical margin). Model performance was assessed for the Sarculator and PERSARC calculators by examining discrimination (C-index) and calibration (calibration plots and observed-expected statistic; O/E-statistic). Internal-external cross-validation by different regions was performed to evaluate the generalizability of the model.
UNASSIGNED: A total of 507 patients with primary MPNSTs were included from 11 centers in 7 regions. During follow-up (median 8.7 years), 211 patients died. The C-index was 0.60 (95% CI 0.53-0.67) for both Sarculator and PERSARC. The MPNST-specific model had a pooled C-index of 0.69 (95%CI 0.65-0.73) at validation, with adequate discrimination and calibration across regions.
UNASSIGNED: The MPNST-specific MONACO model can be used to predict 3-, 5-, and 10-year OS in patients with primary MPNST who underwent macroscopically complete surgical resection. Further validation may refine the model to inform patients and physicians on prognosis and support them in shared decision-making.
UNASSIGNED: This is a retrospective multicenter cohort study of patients with MPNST from 11 secondary or tertiary centers in The Netherlands, Italy and the United States of America. All patients diagnosed with primary MPNST who underwent macroscopically complete surgical resection from 2000 to 2019 were included in this study. A multivariable Cox proportional hazard model for OS was estimated with prespecified predictors (age, grade, size, NF-1 status, triton status, depth, tumor location, and surgical margin). Model performance was assessed for the Sarculator and PERSARC calculators by examining discrimination (C-index) and calibration (calibration plots and observed-expected statistic; O/E-statistic). Internal-external cross-validation by different regions was performed to evaluate the generalizability of the model.
UNASSIGNED: A total of 507 patients with primary MPNSTs were included from 11 centers in 7 regions. During follow-up (median 8.7 years), 211 patients died. The C-index was 0.60 (95% CI 0.53-0.67) for both Sarculator and PERSARC. The MPNST-specific model had a pooled C-index of 0.69 (95%CI 0.65-0.73) at validation, with adequate discrimination and calibration across regions.
UNASSIGNED: The MPNST-specific MONACO model can be used to predict 3-, 5-, and 10-year OS in patients with primary MPNST who underwent macroscopically complete surgical resection. Further validation may refine the model to inform patients and physicians on prognosis and support them in shared decision-making.
摘要：
■本研究旨在评估当前可用的风险计算器在恶性外周神经鞘瘤（MPNST）患者队列中的表现，并创建MPNST特异性预后模型，包括类型特异性预测因子总生存期（OS）。
■这是一项来自荷兰11个二级或三级中心的MPNST患者的回顾性多中心队列研究，意大利和美利坚合众国。从2000年到2019年，所有诊断为原发性MPNST的患者均接受了宏观完整的手术切除。用预先指定的预测因子(年龄，grade,尺寸,NF-1状态，triton状态，深度,肿瘤位置,和手术切缘)。通过检查判别（C指数）和校准（校准图和观察到的预期统计量；O/E统计量），评估了Sarculator和PERSARC计算器的模型性能。通过不同区域的内部-外部交叉验证来评估模型的可泛化性。
■共纳入来自7个地区11个中心的507例原发性MPNSTs患者。在随访期间（中位数为8.7年），211名患者死亡。Sarculator和PERSARC的C指数均为0.60（95％CI0.53-0.67）。MPNST特异性模型在验证时的合并C指数为0.69(95CI0.65-0.73)，在不同地区进行充分的区分和校准。
■MPNST特定的MONACO模型可用于预测3-，5-,接受宏观完整手术切除的原发性MPNST患者的10年OS。进一步的验证可以改进模型，以告知患者和医生预后，并支持他们共同决策。
■这是一项来自荷兰11个二级或三级中心的MPNST患者的回顾性多中心队列研究，意大利和美利坚合众国。从2000年到2019年，所有诊断为原发性MPNST的患者均接受了宏观完整的手术切除。用预先指定的预测因子(年龄，grade,尺寸,NF-1状态，triton状态，深度,肿瘤位置,和手术切缘)。通过检查判别（C指数）和校准（校准图和观察到的预期统计量；O/E统计量），评估了Sarculator和PERSARC计算器的模型性能。通过不同区域的内部-外部交叉验证来评估模型的可泛化性。
■共纳入来自7个地区11个中心的507例原发性MPNSTs患者。在随访期间（中位数为8.7年），211名患者死亡。Sarculator和PERSARC的C指数均为0.60（95％CI0.53-0.67）。MPNST特异性模型在验证时的合并C指数为0.69(95CI0.65-0.73)，在不同地区进行充分的区分和校准。
■MPNST特定的MONACO模型可用于预测3-，5-,接受宏观完整手术切除的原发性MPNST患者的10年OS。进一步的验证可以改进模型，以告知患者和医生预后，并支持他们共同决策。
