Recurrent malignant peripheral nerve sheath tumor of the parietal scalp

Malignant peripheral nerve sheath tumors (MPNSTs) are a rare and aggressive subtype of sarcomas defined by their neural origin. Head and neck manifestations are particularly uncommon. Challenges exist in diagnosis, management, and recurrence. Achieving local control, particularly in the head and neck region, is difficult. We present a patient with a rapidly enlarging MPNST on the right parietal calvarium shortly after resection of a right vagus nerve MPNST. Recommendation was made for excision and reconstruction with a local advancement flap followed by radiation therapy. Local control with good aesthetic outcome was achieved by applying recommended surgical and oncologic principles.