A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report.

Choi, Dong Il; Seo, Dong Kuk; Jo, Woo Sung; Chung, Chul Hoon; Cho, Seong Jin

Case Report

Journal of the Korean Society of Plastic and Reconstructive Surgeons 2006;33(6):761-763.
Published online November 1, 2006.

A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report.

Dong Il Choi, Dong Kuk Seo, Woo Sung Jo, Chul Hoon Chung, Seong Jin Cho

¹Department of Plastic and Reconstructive Surgery, Hallym University, Seoul, Korea. dkseo@hallym.or.kr
²Department of Pathology, College of Medicine, Hallym University, Seoul, Korea.

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.

Keywords: Malignant peripheral nerve sheath tumor; Neurofibromatosis