A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1.
- Author:
Min Ju KANG
1
;
Hoon KANG
;
Hyung Ok KIM
;
Young Min PARK
Author Information
- Publication Type:Case Report
- Keywords: Malignant peripheral nerve sheath tumor; Neurofibromatosis type 1
- MeSH: Adult; Biopsy; Buttocks; Humans; Lymph Nodes; Neoplasm Metastasis; Nerve Sheath Neoplasms; Neurilemmoma; Neurofibromatoses; Neurofibromatosis 1; Neurofibrosarcoma; Peripheral Nerves; Prognosis; Recurrence
- From:Annals of Dermatology 2008;20(1):32-36
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
