Malignant peripheral nerve sheath tumor of the head and neck in the pericarotid plexiform: a case report of a rare disease entity

Abstract Malignant peripheral nerve sheath tumors (MPNST) are rare high-grade sarcomas that arise from cells of the peripheral nerve sheaths. They are considered as one of the most aggressive malignant lesions in the head and neck region, with a notably high rate of local recurrence. In the current case report, we described A 28-year-old female known to have neurofibromatosis type 1 (NF1) with a rare case of MPNST in the right sided neck and reviewed the relevant literature. Computed tomography (CT) scan of the head and neck with contrast was initially obtained and showed right sided soft tissue neck mass that was predominantly cystic. Head and neck magnetic resonance imaging (MRI) with contrast demonstrated a large right pericarotid plexiform heterogeneously enhancing tumor. Subsequently surgical excision of the mass under general anesthesia was done. Histopathology of the mass was reported to be malignant peripheral nerve sheath tumor (low grade). The patient was kept on regular follow-up in the head and neck combined clinic, and the surgical site healed well in six weeks, with no sign of local recurrence or metastasis.