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KMID : 0032220080200010032
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Annals of Dermatology
2008 Volume.20 No. 1 p.32 ~ p.36
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A Case of Malignant Peripheral Sheath TumorArising from Neurofibromatosis Type 1
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Kang Min-Ju
Park Young-Min
Kim Hyung-Ok
Kang Hoon
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Abstract
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Malignant peripheral nerve sheath tumor (MPNST) is a term encompassing tumors previously diagnosed as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and neurofibrosarcoma The occurrence rate of MPNST in neurofibromatosis type 1 patients is known to be about 4.6%. Tumors occurring in this particular group have a worse prognosis in that they occur at an earlier age, are more centrally located, tend to be of a larger size and show more metastases and recurrences. We present a typical case of MPNST in a 36-year-old man with NF type 1, which occurred on the left buttock. A PET-CT showed findings of possible inguinal lymph node metastasis and a lymph node biopsy confirmed the diagnosis. The patient was treated with wide surgical resection and is undergoing adjuvant radiation therapy.
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KEYWORD
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Malignant peripheral nerve sheath tumor, Neurofibromatosis type 1
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