An Uncommon Case of Neurofibromatosis Type 2: A Tribute to the Intracranial Calcifications
A 34-year-old man with a complaint of mild hearing loss in the left ear was referred to our department for temporal bone CT. His family history was unremarkable. Temporal bone CT [Figure –], showed subependymal calcification at the foramen of Monro [], choroid plexus calcifications within the lateral ventricles [], a small calcified lesion adjacent to the right cavernous sinus [], and a partially calcified midline mass adjacent to the fourth ventricle []. These findings were found highly suspicious for NF2, although no prominent mass was seen within the cerebellopontine cisterns or internal acoustic canals. Thus, further imaging with MRI was recommended. Cerebral MRI with intravenous contrast administration showed no major findings except an enhancing mass at the posterior aspect of the fourth ventricle . Inner ear MRI with the aid of fast imaging employing steady state acquisition (FIESTA) [] and contrast enhanced T1-weighted sequences with thin slices [] revealed bilateral small intracanalicular lesions consistent with schwannomas. In addition, the small calcified lesion adjacent to the right cavernous sinus showed contrast enhancement indicating a meningioma. Spinal MRI [Figure and ], which was performed to evaluate possible additional tumors, showed a mass causing focal cord expansion at C5-C6 level [] and multiple enhancing small intramedullary lesions [] consistent with ependymomas in the cervical cord. All these findings supported the final diagnosis of NF2. Posterior fossa mass was operated and diagnosed as WHO Grade 2 ependymoma histopathologically [].