Recurring Intracranial Malignant Peripheral Nerve Sheath Tumor: Case Report and Systematic Review of the Literature

BACKGROUND AND IMPORTANCE: To report the clinical presentation and management of an intracranial frontoparietal malignant peripheral nerve sheath tumor (MPNST) and its recurrence in a 6-year-old girl, along with a systematic review of the literature. CLINICAL PRESENTATION: A previously healthy 6-year-old girl presented with severe signs of increased intracranial pressure. Magnetic resonance imaging (MRI) revealed a large heterogeneously enhancing mass at the right frontoparietal convexity. The tumor was completely removed, the histological diagnosis was MPNST, and the patient underwent adjuvant radio- and chemotherapy. Fifteen months later, MRI revealed a small local recurrence. After upfront chemotherapy, the recurrence was removed in a radical, ie, true oncological, neuronavigationally guided, en bloc resection, including approximately 1 cm surrounding gray and white matter and overlying dura mater. Neuropathological examination revealed tumor-free margins. The patient again received adjuvant chemotherapy. Four years after diagnosis and 20 months after cessation of adjuvant therapy, there are no signs of tumor recurrence. The literature search resulted in 17 cases of intracranial MPNSTs not associated with cranial nerves. Despite macroscopically complete resection in many cases and adjuvant radio- and chemotherapy, overall survival was poor, with only 5 patients still alive upon publication ( including the current patient). CONCLUSION: Intracranial MPNSTs not associated with cranial nerves are extremely rare and highly malignant tumors with poor overall survival, probably because of infiltrating growth into surrounding brain tissue. We therefore advocate consideration of non-conventional true oncological en bloc resection when approaching this rare tumor or its recurrence.