Malignant Peripheral Nerve Sheath Tumor of Central Origin: Case Report and Review of the Literature

Abstract

This case report describes a rare tumor, Malignant Peripheral Nerve Sheath Tumor of Central Nervous System Origin, previously Malignant Schwannoma of Central Nervous System. This is the sixth case reported in literature, and the patient was a twelve-year old boy. This is the first case associated to neurofibromatosis type 1 (von Recklinghausen’s disease). In October 1999, the patient went for a medical visit with the following complaints: headache, nausea, signs of facial paralysis, and hilaric conduct. Imaging studies showed a tumor in the frontal left lobe, a meningioma-like tumor. The pathologic study revealed fusocelular malignant characteristics and the imunochemistry study confirmed the true diagnosis. Afterwards it was established radiotherapy. Thirty days after the end of the treatment, there was recurrence to the scalp (on the surgical scar), so it was performed chemotherapy according to the Pediatric Oncology Group Protocol for High Grade Sarcomas. The boy finished his treatment on March 2001 without clinical symptons or imaging signs of active disease.