A Case of Malignant Peripheral Nerve Sheath Tumor of the Neck Associated with Neurofibromatosis Type I.
10.3342/kjorl-hns.2010.53.10.657
- Author:
Dong Hwal KIM
1
;
Sung Su LEE
;
Seok Hee LEE
;
Dong Wook LEE
Author Information
1. Department of Otolaryngology-Head and Neck Surgery, Chungbuk National University College of Medicine, Cheongju, Korea. dwlee@chungbuk.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis 1;
Nerve sheath neoplasms
- MeSH:
Female;
Head;
Humans;
Neck;
Nerve Sheath Neoplasms;
Neurilemmoma;
Neurofibroma;
Neurofibromatoses;
Neurofibromatosis 1;
Peripheral Nerves;
Sarcoma;
Skin Abnormalities;
Young Adult
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2010;53(10):657-660
- CountryRepublic of Korea
- Language:Korean
-
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.
