A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report.
- Author:
Dong Il CHOI
1
;
Dong Kuk SEO
;
Woo Sung JO
;
Chul Hoon CHUNG
;
Seong Jin CHO
Author Information
1. Department of Plastic and Reconstructive Surgery, Hallym University, Seoul, Korea. dkseo@hallym.or.kr
- Publication Type:Case Report
- Keywords:
Malignant peripheral nerve sheath tumor;
Neurofibromatosis
- MeSH:
Biopsy;
Follow-Up Studies;
Humans;
Middle Aged;
Neurilemmoma;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1;
Peripheral Nerves*;
Peripheral Nervous System Neoplasms;
Recurrence
- From:Journal of the Korean Society of Plastic and Reconstructive Surgeons
2006;33(6):761-763
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. METHODS: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. RESULTS: There was no evidence of recurrence of tumor for 19 months of follow-up examination. CONCLUSION: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.
