Presentation
Long standing back pain with progressive inability to walk.
Patient Data
Avidly and homogenously enhancing bilateral cerebellopontine angle (CPA) masses, that widened the bilateral porus acusticus internus, similar avidly enhancing mass in the right jugular foramen, avidly enhancing intramedullary lesion at the level of C3-C4, avidly enhancing mass in the left C5-C6 neural exit foramina that compresses the cord and displaces it to the right, intradural extramedullary lesion in the right D5 and D6 levels with severe compression and displacement of the cord, and multiple avidly enhancing intradural extramedullary lesions in the lumbar spinal canal.
The constellation of findings is characteristic of bilateral vestibular schwannoma and schwannomatosis in the remainder neural axis with intramedullary lesion in the cervical cord representing either meningioma or ependymoma.
Case Discussion
Central neurofibromatosis aka neurofibromatosis type 2 is a genetic disorder associated with multiple central and peripheral nervous system tumors. In contrast to neurofibromatosis type 1, it has less cutaneous manifestation. This patient fulfills the criteria for the diagnosis of NF2, that is bilateral vestibular schwannoma. Moreover, there is also NF2 related schwannomatosis and probable ependymoma or meningioma.
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