Abstract

Malignant peripheral nerve sheath tumour (MPNST) is a rare, albeit well-described, complication of neurofibromatosis. We report a 58-year-old patient with known neurofibromatosis Type 1 (NF-1) who presented with an aggressive recurrent malignant peripheral nerve sheath tumour and spinal cord compression 5 weeks after undergoing tumour excision with thoracic (T)6–7 laminectomy. The case and literature review are instructive to those following NF-1 patients with regards to screening for, and management of, malignant conversion of plexiform neurofibroma.

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