title: Comparative Analysis of Head and Neck and Non-Head and Neck Malignant Peripheral Nerve Sheath Tumors.
abstract: OBJECTIVE
  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).
  
  
  METHODS
  Analysis of population-based tumor registry.
  
  
  METHODS
  Academic medical center.
  
  
  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.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Tapan D
authors.initials: TD
authors.lastname: Patel
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Kevin
authors.initials: K
authors.lastname: Shaigany
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Christina H
authors.initials: CH
authors.lastname: Fang
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Richard Chan
authors.initials: RC
authors.lastname: Park
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Soly
authors.initials: S
authors.lastname: Baredes
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, New Jersey, USA Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA Department of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey, USA jean.anderson.eloy@gmail.com.
authors.forename: Jean Anderson
authors.initials: JA
authors.lastname: Eloy
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009442:Neurilemmoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D016019:Survival Analysis; D015996:Survival Rate; D055815:Young Adult
keywords: SEER; comparative analysis; disease-specific survival; epidemiology; head and neck neoplasm; head and neck sarcomas; malignant peripheral nerve sheath tumor; peripheral nerve sheath tumor; sarcomas; survival analysis