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

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.

Keywords

peripheral nerve sheath tumor malignant peripheral nerve sheath tumor head and neck neoplasm comparative analysis SEER disease-specific survival head and neck sarcomas epidemiology survival analysis sarcomas

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References

Bailet

Abemayor

Andrews

Rowland

Dawson

. Malignant nerve sheath tumors of the head and neck: a combined experience from two university hospitals. Laryngoscope. 1991;101:1044-1049.

Mullins

Hackman

. Malignant peripheral nerve sheath tumors of the head and neck: a case series and literature review. Case Rep Otolaryngol. 2014;2014:368920.

Zou

Smith

Liu

. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249:1014-1022.

Peng

Grogan

Wang

. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151:627-633.

Farid

Demicco

Garcia

. Malignant peripheral nerve sheath tumors. Oncologist. 2014;19:193-201.

Anghileri

Miceli

Fiore

. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006;107:1065-1074.

Minovi

Basten

Hunter

Draf

Bockmühl

. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck. 2007;29:439-445.

Porter

Prasad

Foster

Dall

Birch

Grimer

. Survival in malignant peripheral nerve sheath tumours: a comparison between sporadic and neurofibromatosis type 1-associated tumours. Sarcoma. 2009;2009:756395.

Eeles

Fisher

A’Hern

. Head and neck sarcomas: prognostic factors and implications for treatment. Br J Cancer. 1993;68:201-207.

10.

Eryilmaz

Varsak

Gul

Etli

. Second primary malignant peripheral nerve sheath tumor of the paranasal sinuses after nasopharynx carcinoma. J Craniofac Surg. 2014;25:2074-2076.

11.

Mushi

Winter

. Management of an incidental malignant peripheral nerve sheath tumour in the parapharyngeal space. J Laryngol Otol. 2013;127:104-106.

12.

Shan

. Malignant peripheral nerve sheath tumours in the head and neck region: retrospective analysis of clinicopathological features and treatment outcomes. Int J Oral Maxillofac Surg. 2014;43:924-932.

13.

Wong

Hirose

Scheithauer

Schild

Gunderson

. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys. 1998;42:351-360.

14.

Greager

Reichard

Campana

DasGupta

. Malignant schwannoma of the head and neck. Am J Surg. 1992;163:440-442.

15.

Touil

Briki

Karray

Bahri

. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. Eur Ann Otorhinolaryngol Head Neck Dis. 2015;132:93-95.

16.

Kolberg

Holand

Agesen

. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 2013;15:135-147.

17.

Loree

North

Jr Werness

Nangia

Mullins

Hicks

Jr . Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors. Otolaryngol Head Neck Surg. 2000;122:667-672.

18.

Vege

Chinoy

Ganesh

Parikh

. Malignant peripheral nerve sheath tumors of the head and neck: a clinicopathological study. J Surg Oncol. 1994;55:100-103.

19.

Hagel

Zils

Peiper

. Histopathology and clinical outcome of NF1-associated vs sporadic malignant peripheral nerve sheath tumors. J Neurooncol. 2007;82:187-192.

20.

Schmidt

Yick

Boghani

Eloy

Liu

. Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases. Neurosurg Focus. 2013;34:E5.

21.

Huson

Compston

Clark

Harper

. A genetic study of von Recklinghausen neurofibromatosis in south east Wales: I. Prevalence, fitness, mutation rate, and effect of parental transmission on severity. J Med Genet. 1989;26:704-711.

22.

Hrehorovich

Franke

Maximin

Caracta

. Malignant peripheral nerve sheath tumor. Radiographics. 2003;23:790-794.

23.

LaFemina

Qin

Moraco

. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol. 2013;20:66-72.