Neurofibromatois type 2

Case contributed by Andrew Lawson
Diagnosis probable

Presentation

Headaches and right sided hearing loss.

Patient Data

Age: 50
Gender: Female
mri

Small homogeneously enhancing mass, measuring 14 x 6 mm, arising from within the right internal acoustic canal. The mass expands the internal acoustic meatus, extends along the course of the vestibulocochlear nerve and just projects into the cerebello-pontine angle where it does not contact the brainstem or cerebellar peduncle. The left internal acoustic meatus is normal.

Further homogeneously enhancing lesion centered, and contained within, the boundaries, of the fourth ventricle measuring 11 x 9 x 19 mm ( AP, transverse, CC ). It does not result in obstruction to CSF flow. The temporal horns and lateral ventricles are not dilated. The mass returns intermediate homogeneous T2 signal and does not exhibit restricted diffusion. No surrounding FLAIR signal abnormality or substantial mass effect.

Deep white matter T2 hyperintensities are consistent with the patient's age. No remote extra axial lesions. The optic nerves and optic chiasm are unremarkable.

Conclusion:

1. Right acoustic neuroma with no mass effect on the CP angle.

2. Well circumscribed enhancing lesion contained within the fourth ventricle. The differential for this would be either an ependymoma or subependymoma.

3. The presence of an acoustic neuroma and ependymoma raises the possibility of neurofibromatosis type II.

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