Analysis of Survival Differences Between Cutaneous and Subcutaneous Malignant Peripheral Nerve Sheath Tumors.

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are clinically aggressive soft tissue sarcomas that can present as cutaneous or subcutaneous based tumors that are commonly associated with neurofibromatosis type 1. Historically, these tumors have poor outcomes. Previously, no study has compared survival of cutaneous versus subcutaneous MPNSTs. This study aims to investigate the difference in overall survival (OS) among cutaneous MPNSTs, subcutaneous MPNSTs of the head and neck, and subcutaneous MPNSTs of the trunk and extremities. Nine hundred eighteen patients were included in this retrospective study using the Surveillance, Epidemiology, and End-Results (SEER-9) database with primary cutaneous or subcutaneous MPNSTs from 1975 to 2016. OS was calculated using cox proportional hazard models for each group. No significant difference was revealed in OS between cutaneous or subcutaneous MPNSTs, regardless of location. Factors associated with decreased OS included advanced age, higher grade, and nondefinitive surgical modality. This study results implies that unlike other soft tissue sarcomas, cutaneous presentation does not improve OS in patients with MPNSTs compared with their subcutaneous counterparts.