Neurofibromatosis type 2

Case contributed by Dr Hani Makky ALSALAM

Presentation

Headache, swelling over the calvarium and right-sided ptosis.

Patient Data

Age: 15 years
Gender: Male
MRI

Brain

A large right frontal extra-axial lesion is noted with homogeneous texture, homogeneous postcontrast enhancement and diffusion restriction. It has a wide dural-base with smooth scalloping of the inner table. It exerts mass effect in the form of compression of both frontal lobes, markedly on the related right side. CSF cleft is noted, confirming extra-axial location. It has prominent feeding arteries. Features are characteristic for right frontal large convexity meningioma.

Right cerebellopontine cistern mass lesion with intense postcontrast enhancement, also suggestive of right CPA meningioma. 

Sagittal postcontrast T1WI sequence shows posterior cervical canal dural-based extra-axial lesion with homogeneous postcontrast enhancement. It is extending to the foramen magnum compressing the proximal cervical cord and cervicomedullary junction, suggestive of spinal meningioma.

X-ray

Widened unfused coronal suture, may suggest increased intracranial tension.

CT

Brain

The right frontal extra-axial lesion shows internal calcifications and related skull hyperostosis of the right frontal bone, related to its base. 

Right CPA lesion is calcified, mostly calcified meningioma.

DSA (angiography)

The right frontal lesion prior to embolization.

Case Discussion

Brain CT and MRI revealed multiple extra-axial masses with the largest over the right frontal convexity (pathologically proven meningioma) the rest of the lesions are seen at the right cerebellopontine angle and upper cervical region, subsequently the patient was confirmed to have NF2.

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Case information

rID: 8953
Published: 11th Mar 2010
Last edited: 13th Dec 2019
Tag: brain, nf2
Inclusion in quiz mode: Included

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