2018 Volume 32 Issue 3 Pages 279-284
A 72-year-old Japanese man with neurofibromatosis type 1 presented with a subcutaneous nodule on the right inguinal region two months ago. Histopathological findings showed increased numbers of atypical S100 protein-positive spindle cells. Positron emission tomography-computed tomography revealed 18F-fluorodeoxyglucose (FDG) accumulation in the right inguinal lymph nodes. The patient was referred to us under the tentative diagnosis of a malignant peripheral nerve sheath tumor. Physical examination revealed not only numerous lesions of neurofibroma and café-au-lait spots on his trunk and extremities, but also an ulcerative lesion with black and depigmented macules on his right big toe. The patient underwent surgical resection and total lymph node dissection. Based on the histopathological findings, the patient was diagnosed with metastatic malignant melanoma.[Skin Cancer (Japan) 2017 ; 32 : 279-284]