Yuan Zhennan, Xu Libin, Zhao Zhenguo, Xu Songfeng, Zhang Xinxin, Liu Ting, Zhang Shuguang, Yu Shengji
Department of Orthopaedics, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Oncotarget. 2017 Jul 4;8(62):104785-104795. doi: 10.18632/oncotarget.18975. eCollection 2017 Dec 1.
To investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors (MPNST).
A total of 159 patients with MPNST were enrolled in the study. The ratio of male to female was 1.04 to 1. The median age was 40 (range: 5-76) years at the time of diagnosis. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. The median follow-up period was 31.0 (range: 2.0-199.0) months. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival ( < 0.05 for both). 3- and 5-year tumor-free survival rates for 140 completely resected patients were 40.0% and 34.0%, respectively. Multivariate analysis showed that AJCC stage, S-100 and Ki67 staining were independent factors of tumor-free survival ( < 0.05 for all).
The clinical data of MPNST patients who were treated at Cancer Institute and Hospital, Chinese Academy of Medical Science from January 1999 to January 2016 was retrospectively reviewed.
MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. This had been largest documented retrospective study of MPSNT among Chinese populations. Some characteristics were different from those of foreign populations which may suggest the specificity of Chinese patients.
探讨恶性外周神经鞘膜瘤（MPNST）的临床病理特征及预后。
本研究共纳入159例MPNST患者。男女比例为1.04:1。诊断时的中位年龄为40岁（范围：5 - 76岁）。3年和5年总生存率分别为50.0%和43.0%。中位随访期为31.0个月（范围：2.0 - 199.0个月）。多因素分析显示，美国癌症联合委员会（AJCC）分期和S - 100是影响总生存的独立因素（两者均P<0.05）。140例完全切除患者的3年和5年无瘤生存率分别为40.0%和34.0%。多因素分析显示，AJCC分期、S - 100和Ki67染色是无瘤生存的独立因素（均P<0.05）。
回顾性分析1999年1月至2016年1月在中国医学科学院肿瘤医院接受治疗的MPNST患者的临床资料。
MPNST是一种侵袭性很强、预后较差的肿瘤，本研究可能有助于预后评估及治疗决策。这是中国人群中关于MPNST最大规模的文献记载的回顾性研究。一些特征与国外人群不同，这可能提示中国患者的特异性。
