Clinicopathological Features And Prognosis Of Malignant Peripheral Nerve Sheath Tumor:A Retrospective Study Of 140 Cases

Objective To investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors(MPNST).Methods We retrospectively reviewed the clinical data of MPNST patients who were treated at Cancer Institute&Hospital,Chinese Academy of Medical Science from January 1999 to January 2016.A total of 140 patients with 66 male and 74 female with MPNST were enrolled in the study.The median age was 40 years at the time of diagnosis.Survival analysis were estimitated by Kaplan-Meier method and Log rank test.Multivariate analysis were estimitated by Cox proportional hazards regression model.Results The median follow-up period was 43.0 months.The 3-and 5-year overall survival(OS)rates were 56.4%and 48.6%,respectively.The 3-year local recurrence(LR)rate and distant metastasis(DM)rate were 42.6%and 49.3%,respectively.Univariable analysis showed that the tumor location,the depth,AJCC stage,S-100,radiotherapy and margin status affected 5-year OS rate(all P<0.05).The tumor location,the depth,AJCC stage,S-100,Ki-67 staining,margin status,radiotherapy and chemotherapy affected 3-year LR rate(all P<0.05).The tumor location,the size,the depth,AJCC stage,S-100,Ki-67 staining and margin status affected 3-year DM rate(all P<0.05).Multivariate analysis showed that the tumor AJCC stage,S-100 were independent factors for 5-year OS rate(all P<0.05).The tumor location,Ki-67 staining and chemotherapy were independent factors for LR(all P<0.05)while the depth,the size,AJCC stage,and Ki-67 staining were independent factors of DM(all P<0.05).Conclusions MPSNT is an aggressive tumor with poor prognosis.Multiple factors were identified in this study.Patients with the tumor of advanced AJCC stage,negative S-100 usually have a low 5-year overall survival rate.Patients with the tumor located in the head and neck,Ki-67 staining ≥ 20%and without chemotherapy had a higher tendency of local recurrence.Poor prognosis factors for DM were the deep location,size>5cm,advanced AJCC stage,positive margin and Ki-67 staining ≥ 20%.