악성 말초 신경 초종의 원격 재발

PMalignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma of ectomesenchymal origin. We experienced a case of metastatic MPNST from oral cavity onto skull with aggravation of malignant phenotype, and reported it with literature review. A 75-year-old woman was presented with a 3-months history of growing and indurate mass on her frontal skull. The patient underwent bifrontal craniectomy with safety margin from the lesion. Two years before, she had undergone trans-oral wide resection and anterior partial maxillectomy to extirpate the MPNST at oral cavity. No adjuvant therapy was added at that time. Histopathologic examination showed an poorly differentiated sarcoma. The tumor demonstrated palisade alignment, necrosis, cellular pleomorphism and increased mitosis of tumor cells. And their immunohistochemical studies showed positive in S-100, CK, CD68, Vimentin, Actin, and CD99. She received postoperative adjuvant radiation therapy. It was obvious that the tumor had changed toward more malignant phenotype. We strongly recommend a careful follow-up of remote area even after the wide resection in MPNST patient.