J Korean Soc Plast Reconstr Surg.  2006 Nov;33(6):761-763.

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

• Choi DI
• Seo DK
• Jo WS
• Chung CH
• Cho SJ

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.

Keyword

Malignant peripheral nerve sheath tumor; Neurofibromatosis

MeSH Terms

Biopsy
Follow-Up Studies
Humans
Middle Aged
Neurilemmoma
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1
Peripheral Nerves*
Peripheral Nervous System Neoplasms
Recurrence