Malignant peripheral nerve sheath tumor of the right forearm: Case report

Malignant peripheral nerve sheath tumor (MPNST), also called malignant schwannoma, neurofibrosarcoma and neurogenic sarcoma, is a malignant neoplastic lesion originating in the Schwann cells of the sheath lining of peripheral nerves.

A 2-year-old, 7-month-old female patient, was admitted to our clinic due to progressive enlargement of the right forearm, physical examination revealed a tumor on the right forearm, painless, fixed to adjacent structures, hard, with erythema and swelling in the upper part of the lesion, complete surgical excision of the tumor with left inguinal graft was performed, covering the entire resected surgical defect. The pathology report was a malignant peripheral nerve sheath tumor. The patient was discharged with good clinical evolution, the MPNST was low grade so no adjuvant treatment based on radiotherapy or chemotherapy was administered.

These malignant tumors are recognized for being aggressive, for high rates of local recurrence and distant metastases, constitute approximately 5 % to 10 % of all soft tissue sarcomas, and arise mainly from peripheral nerve sheath components, with poor prognosis.

This case shows the clinical manifestations and complications that can be expected with these tumors, as well as their reconstructive treatment with adequate esthetic and functional results in young patients.