A Case Report of a Malignant Peripheral Nerve Sheath Tumor of the Oral Cavity in Neurofibromatosis Type 1

الملخص EN

Patients with neurofibromatosis type 1 develop both benign and malignant tumors at an increased frequency.

Most of the malignant peripheral nerve sheath tumors (MPNSTs) are considered as high-grade sarcomas originating from tissues of mesenchymal origin.

It is generally accepted that MPNSTs occur in about 2% to 5% of neurofibromatosis patients.

In this paper, we present a 16-year-old male patient with neurofibromatosis who developed MPNST of the retromolar area.

The mass enlarged rapidly in a period of 6 weeks.

The patient was treated surgically, and a tumor mass with a diameter of 7×6×4 cm was excised, but after 8 months a recurrence was observed at the same site.

The sarcomatous change in a neurofibroma has an extremely poor prognosis, so patients with neurofibromatosis should be closely monitored for a possible malignancy.

A rapid change in size of a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain indicate a possible malignant transformation to MPNST.