Comparative Analysis of Head and Neck and Non–Head and Neck Malignant Peripheral Nerve Sheath Tumors

Authors:
Publisher website
Google Scholar
Abstract
Objectives: Malignant peripheral nerve sheath tumors (MPNSTs) are a group of tumors that arise from peripheral nerves or from the various elements of the nerve sheath, including Schwann cells and perineural fibroblasts. Head and neck MPNSTs (HN‐MPNSTs) are rare, accounting for 8% to 16% of all soft tissue sarcomas. This study analyzes the demographic, clinicopathologic, and survival characteristics of HN‐MPNSTs and establishes comparisons with MPNSTs at other body sites (other‐MPNSTs).Study Design: Analysis of population‐based tumor registry.Setting: Academic medical center.Subjects and Methods: The SEER database (Surveillance, Epidemiology, and End Results; 1973‐2012) was queried for HN‐MPNSTs (324 cases) and other‐MPNSTs (1680 cases). Data were analyzed comparatively with respect to various demographic and clinicopathologic factors. Disease‐specific survival was analyzed with the Kaplan‐Meier model.Results: Mean age at diagnosis for HN‐MPNST was 49.1 years, compared with 46.1 years for other‐MPNSTs (P =. 0169). There was a sex predilection for males in HN‐MPNSTs (60.2%) versus a female predilection for other‐MPNSTs (54.2%; P <. 0001). Average tumor size for HN‐MPNSTs was 4.9 cm, compared with 8.7 cm for other‐MPNSTs (P <. 0001). HN‐MPNSTs were more commonly of low histologic grade types, whereas other‐MPNSTs were mostly of high histologic grade (P =. 0073). HN‐MPNSTs had a higher 5‐year disease‐specific survival than other‐MPNSTs (65.1% vs 57.4%; P =. 0209).Conclusions: HN‐MPNSTs are rare entities. This study represents the largest series of HN‐MPNSTs to date. Although HN‐MPNSTs and other‐MPNSTs share a common histology, there are important clinical differences between the 2 groups.

This publication has 24 references indexed in Scilit: