Spinal cord compression from cutaneous malignant peripheral nerve sheath tumor metastasis in a patient with neurofibromatosis Type 1

Abstract

Background: Neurofibromatosis Type 1 (NF-1) and previous irradiation are two common risk factors that can result in malignant peripheral nerve sheath tumors (MPNSTs), extremely rare soft-tissue sarcomas. Here, a 63-year-old male with NF-1 presented with diffuse spinal metastases from a subcutaneous MPNST. Case Description: A 63-year-old male with NF-1 presented acutely with paraplegia and urinary incontinence. Both CT and MR studies of the thoracic-lumbosacral spine showed multiple metastases from a subcutaneous MPNST. In addition, the patient had a T12 vertebral body pathological fracture. Conclusion: Despite its aggressive behavior, some cases of MPNST can be managed with gross total resection and adjuvant radiotherapy. In addition, in the presence of multiple metastases, chemotherapy may play an additional, although questionable role.