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J Korean Soc Plast Reconstr Surg. 2006 Nov;33(6):761-763. Korean. Case Report.
Choi DI , Seo DK , Jo WS , Chung CH , Cho SJ .
Department of Plastic and Reconstructive Surgery, Hallym University, Seoul, Korea.
Department of Pathology, College of Medicine, Hallym University, Seoul, Korea.

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.

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