Malignant peripheral nerve sheath tumours in the head and neck region: retrospective analysis of clinicopathological features and treatment outcomes

Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcomas. The aim of this study was to assess clinicopathological characteristics and prognostic factors in order to improve the treatment of such tumours in the head and neck region. We performed a retrospective analysis of head and neck MPNST patients in our hospital between 1996 and 2012. Clinical features and pathological findings of these cases (n = 43) were summarized. In addition, prognostic variables were evaluated by univariate and multivariate analyses. The median age of the patients at presentation was 41 years. Surgery was the main treatment approach. Pertinent information regarding the presence of neurofibromatosis type 1 was found in 13 patients (30.2%). Two-thirds of these patients were admitted for a primary tumour (n = 27, 62.8%), while one-third (n = 16, 37.2%) were treated for recurrent neoplasms. The overall survival rate was 46.5%. Multivariable analysis identified tumour size, surgical margins, and postoperative radiotherapy to be independent prognostic factors. MPNST of the head and neck is extremely difficult to manage. Surgery with postoperative radiation may be the optimum choice of treatment for primary head and neck MPNST.