Authors
Abstract
Background and Purpose: Type II neurofibromatosis is a dominant autosomal genetically transferred disease characterized with multiple Schwannoma and classically with bilateral vestibular Schwannoma. Schwannoma is considered as the main symptom and usually originates from cranial nerves (particularly the 8th pair), nerve roots and distal nerves. In this article, one such case is reported.
The Patient: A 20-year-old patient with a history of progressive hearing loss from one year earlier was admitted. The history of the patient indicated a spinal Schwannoma surgery one year before the first visit. No familial history was recorded. On the systemic examination, only a few milk brown spots were observed. On the nervous system examination, low facial sensation along the ophthalmic and maxillary branches of the 5th pair, low OS retina reflex and left ear hearing loss were observed. MR imaging indicated hyper-intense damages in T2 phase from the cerebellar-pons angle, highlighting the mass on the right with the radio-opaque agent. Upon left occipital craniotomy, the calcified rather bleeding tumor underwent resection in the cerebellar-pons area. Pathology results confirmed Schwannoma.
Conclusion: The singleton type II neurofibromatosis is rare and consideration of the disorder seems to be essential in the diagnosis of multiple simultaneous tumors in the central nervous system.
Keywords
)