Objective and importance: Schwannomas occurring in the neuraxis are very rare. Usually, these tumors are benign. Primary malignant intracerebral nerve sheath tumors are extremely rare, with only five documented cases in the international literature. We report one case of a primary malignant intracerebral nerve sheath tumor occurring in the right cerebral peduncle of a 35-year-old man.
Clinical presentation: Magnetic resonance imaging revealed a heterogeneous peripherally enhancing mass of the right cerebral peduncle, surrounded by a small edema.
Intervention: Unlike the five cases previously reported, this is the first time a stereotactic biopsy has been performed, and this is the only patient who responded to cranial radiation therapy for approximately 2 years. When the tumor recurred, a systemic chemotherapy treatment was prescribed. No positive response was seen, and the patient died 29 months after the initial diagnosis.
Conclusion: An accurate diagnosis and planned aggressive treatment seem to be the key elements in the management of the disease.