[Case of jugular foramen schwannoma associated with tuberous sclerosis]

We report a case of jugular foramen schwannoma associated with tuberous sclerosis. A 28-year-old female with tuberous sclerosis presented to Hikone Municipal Hospital with subacute onset of somnolence, ataxic gait and frequent vomiting. Her tuberous sclerosis was diagnosed at the age of 9, when she developed hydrocephalus caused by subependymal giant cell astrocytoma near the foramen of Monro, which was treated by surgical resection and VP shunt placement followed by radiation and chemotherapy. Brain MR images revealed a 3 cm enhancing mass extending from the left jugular foramen to the cerebellopontine cistern, accompanied by perifocal edema of the brain stem and cerebellar hemisphere. The tumor was partially removed via suboccipital craniotomy and histologically diagnosed as a schwannoma. After surgery her symptoms promptly improved and 4 months later she underwent additional stereotactic radiosurgery. No tumor regrowth has been observed at 94 months. Intracranial schwannoma complicating tuberous sclerosis is very rare. Only one other case has been reported in the literature.