A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1.
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.
Department of Dermatology, Kangnam St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Min Ju
MJ
Kang
Department of Dermatology, St. Paul's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Hoon
H
Kang
Department of Dermatology, Kangnam St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Hyung Ok
HO
Kim
Department of Dermatology, Kangnam St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Young Min
0000-0002-3631-0807
YM
Park
Malignant peripheral nerve sheath tumor; Neurofibromatosis type 1