title: Malignant peripheral nerve sheath tumors of the head and neck: Demographics, clinicopathologic features, management, and treatment outcomes.
abstract: OBJECTIVE
  To determine the epidemiology and prognostic indicators in patients with malignant peripheral nerve sheath tumors (MPNST) of the head and neck.
  
  
  METHODS
  The surveillance, epidemiology, and end results registry was reviewed for patients with head and neck MPNST from 1973 to 2011. Study variables included age, sex, race, tumor size, stage at presentation, and treatment modality.
  
  
  RESULTS
  There were 374 cases of head and neck MPNST identified. Mean age at diagnosis was 50.7 y ears; 60.2% of patients were male and 82.6% were white. After diagnosis, 38.8% of patients underwent surgery and radiation therapy and 48.1% underwent surgery alone. Kaplan-Meier analysis demonstrated overall (OS) and disease-specific survival (DSS) of 51% and 67% at 5 years. Multivariate Cox regression analysis showed that age (p=0.030), stage (p=0.002), surgery (p=0.037), and size (p<0.001) were predictors of OS, while stage (p<0.001) and size (p<0.001) were predictors of DSS. For stage I/II cancers, surgery (p=0.011) and size (p=0.010) were predictors of OS, and size (p=0.001) predicted DSS. For stage III/IV cancers, both radiotherapy (p=0.024, p=0.009) and size (p=0.001, p=0.001) predicted OS and DSS. For tumors ⩽5 cm, stage (p=0.031) predicted DSS. For tumors >5 cm, male gender (p=0.005), stage (p=0.001), surgery (p=0.003), and radiotherapy (p=0.050) were determinants of OS, and male gender (p=0.022), stage (p<0.001), and radiotherapy (p=0.002) were determinants of DSS.
  
  
  CONCLUSIONS
  Surgical resection confers survival benefit in patients with early stage MPNST, while radiotherapy improves survival in cases with metastatic disease. Surgery and radiotherapy are prognostically important in patients with tumors >5 cm.
authors.affiliation: Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
authors.forename: Armin
authors.initials: A
authors.lastname: Arshi
authors.affiliation: Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; Head and Neck Cancer Program, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
authors.forename: Bobby A
authors.initials: BA
authors.lastname: Tajudeen
authors.affiliation: Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; Head and Neck Cancer Program, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA. Electronic address: mstjohn@mednet.ucla.edu.
authors.forename: Maie
authors.initials: M
authors.lastname: St John
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D009442:Neurilemmoma / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012307:Risk Factors; D018426:SEER Program; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
keywords: Database; Head and neck cancer; MPNST; Malignant; Nerve; Oral cancer; Peripheral; SEER; Tumor