Korean J Otorhinolaryngol-Head Neck Surg.  2012 Mar;55(3):181-184. 10.3342/kjorl-hns.2012.55.3.181.

A Case of Malignant Peripheral Nerve Sheath Tumor in Parapharyngeal Space

• Lee HS
• Lee CH
• Jin SM
• Lee SH

• KMID: 2277339
• DOI: http://doi.org/10.3342/kjorl-hns.2012.55.3.181

Abstract

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

Keyword

Malignant peripheral nerve sheath tumor; Parapharynx; Neurofibrosarcoma; Malignant schwannoma; Malignant neurilemmoma

MeSH Terms

Deglutition Disorders
Extremities
Head
Headache
Human Body
Humans
Incidence
Middle Aged
Neck
Neurilemmoma
Neurofibromatosis 1
Neurofibrosarcoma
Peripheral Nerves
Recurrence
Sarcoma