Korean J Otorhinolaryngol-Head Neck Surg.  2010 Oct;53(10):657-660. 10.3342/kjorl-hns.2010.53.10.657.

A Case of Malignant Peripheral Nerve Sheath Tumor of the Neck Associated with Neurofibromatosis Type I

• Kim DH
• Lee SS
• Lee SH
• Lee DW

• KMID: 1490041
• DOI: http://doi.org/10.3342/kjorl-hns.2010.53.10.657

Abstract

Neurofibromatosis is an autosomal dominant hereditary disorder characterized by skin abnormalities such as cafe au-lait spots, and soft tissue legion such as generalized subcutaneous neurofibroma. Malignant peripheral nerve sheath tumor is a spindle cell sarcoma that mostly arises in the soft tissue but rarely arises in the head and neck region. Malignant peripheral nerve sheath tumor can develop from the pre-existing neurofibromas or schwannomas, and from the peripheral nerves. They can also occur after radiation therapy. Malignant peripheral nerve sheath tumor is usually associated with neurofibromatosis type I. The authors recently experienced a case of malignant peripheral nerve sheath tumor associated with neurofibromatosis type I in a 20-year-old female patient who presented with a well marginated oval shape mass in the left neck.

Keyword

Neurofibromatosis 1; Nerve sheath neoplasms

MeSH Terms

Female
Head
Humans
Neck
Nerve Sheath Neoplasms
Neurilemmoma
Neurofibroma
Neurofibromatoses
Neurofibromatosis 1
Peripheral Nerves
Sarcoma
Skin Abnormalities
Young Adult