Giant malignant peripheral nerve sheath tumor: Illustrative case and surgical technique

Malignant peripheral nerve sheath tumors (MPNSTs) are one of the most difficult malignant mesenchymal neoplasms to treat. They can arise from a preexisting peripheral nerve sheath or in neurofibromatosis type 1 (NF1) patients. MPNSTs in the head-and-neck region represent approximately 2-6% of all head-and-neck sarcomas and account for 12-19% of all MPNSTs. It has an incidence of 0.001% in the general population, and prognosis is typically poor; only between 20% and 50% of patients survive 5 years after diagnosis. The complete surgical resection is recommended whenever possible.

The authors report the case of a 31-year-old woman with a history of plexiform neurofibroma, who presented with recurrence with disease progression to MPNST, without association with NF1 disease. The aim of tumor resection is symptomatic relief.

These tumors have a high risk of severe complications and even mortality due to various causes. The benefits - including the emotional ones - can be so significant that, despite their malignant lineage, resection and regrowth control remain worthwhile for as long as possible. Four- or six-hand surgery for these tumors can reduce bleeding, operative time, and complications.