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KMID : 0358820060330060761
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Journal of Korean Society of Plastic and Reconstructive Surgeons
2006 Volume.33 No. 6 p.761 ~ p.763
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A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report
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Choi Dong-Il
Seo Dong-Kook
Cho Woo-Sung
Chung Chul-Hoon
Cho Seong-Jin
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Abstract
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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.
Conclusions: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.
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KEYWORD
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Malignant peripheral nerve sheath tumor, Neurofibromatosis
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