Malignant Transformation of Tentorial Schwannoma

Schwannomas are benign tumors derived from Schwann cells, usually seen in the peripheral nervous system. 6.8%-8.0% of all intracranial tumors are accounted by intracranial schwannomas. 90% of these arise from the eighth cranial nerve, less frequently from the fifth, seventh, and twelfth cranial nerves. Schwannomas of the central nervous system [CNS] not arising from cranial nerves are rare. The genesis of these schwannomas in these unusual locations is still controversial since Schwann cells are not a natural component of the CNS. Only 18 tentorial schwannoma [TS] cases have been reported. Interestingly, this is the first reported case of a tentorial schwannoma transforming into a malignant peripheral nerve sheath tumor [MPNST]. We present the case of a 45-year-old gentleman with a giant TS with supra- and infratentorial growth, without any features of neurofibromatosis, and unusual recurrence on follow-up.