Neurofibromatosis type 2

Case contributed by Dr Imran Ahmad Khan


Seizures and left upper limb weakness for 5 hours with loss of consciousness. Past history of brain tumor, operated on 8 years prior.

Patient Data

Age: 25 years
Gender: Female

Neurofibromatosis Type II with multiple CNS neoplasms.


Heterogeneous moderately enhancing masses are seen in bilateral CPA cisterns extending into the internal acoustic meatuses, consistent with bilateral acoustic schwannomas

Moderately enhancing dural based extra-axial masses are seen in interhemispheric fissure in frontal and high parietal regions attached to the falx cerebri, indicative of multiple meningiomas.

An irrregular moderately enhancing intramedullary mass is seen in cervical spinal cord at C4 level. There is associated focal enlargement of the cord. Features are indicative of intramedullary ependymoma.

A large irregular lobulated mass is seen in right parietal region with marked perilesional edema. The mass is of intermediate signal intensity on T1,T2 and FLAIR and shows restricted diffusion on DWI. It shows moderate to intense post contrast enhancement. Foci of low signal instensity on all sequences are seen in the mass indicative of calcifications or hemosiderin deposition (old hemorrhage). Differentials include supratentorial ependymomaprimitive neuroectodermal tumor (PNET) and lymphoma.

Case Discussion

The study reveals bilateral acosutic schwannomas which are highly suggestive of neufibromatosis type II. Neurofibromatosis type II is also known as MISME syndrome for its association with multiple inherited Schwannomas, meningiomas and ependymomas, all of which are clearly delineated in this case. This patient has concurrent bilateral acoustic schwannomas, a cervical intramedullary ependymoma and falcine meningiomas. The large mass in the right parietal lobe can be a supratentorial ependymoma, priminitive neuro-ectodermal tumor or lymphoma. However, association with neurofibromatosis II and restricted diffusion on DWI strongly favors the diagnosis of supratentorial ependymoma.

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