A Malignant Peripheral Nerve Sheath Tumor on the Right Elbow

A 37-year-old man presented with an extremely large tumor (15×12×9cm) over his right elbow that had developed over a period of 6 months. A physical examination revealed multiple tumors in the bilateral limbs, axilla, and back that had developed over the previous 10 years. Computed tomography chest scans revealed multiple nodules in both lungs, indicating metastasis. The patient underwent wide excision of the tumor on his right elbow, which was diagnosed as a stage IV malignant peripheral nerve sheath tumor (MPNST). The patient received radiotherapy for local control of the elbow tumor and chemotherapy for the multiple lung metastatic lesions. The patient died 7 months after surgery due to multiple lung metastasis and respiratory failure. A MPNST is highly aggressive and approximately 50% of cases are associated with neurofibromatosis type 1. MPNST cases have an overall 5-year survival rate of around 34%.