Cervical and acoustic schwannomas suggesting neurofibromatosis type 2

Case contributed by Craig Hacking
Diagnosis certain

Presentation

Left neck pain and paresthesia

Patient Data

Age: 60 - 65 years
Gender: Female

Dumbbell shaped mass widens the left C2/3 neural exit foramen and is iso- to high heterogeneous T2 and iso- to low T1 signal with mildly heterogeneous enhancement. Maximal dimensions are 3 x 1.7 x 2.8 cm. The mass extends to the central spinal canal with significant displacement of the spinal cord towards the right, but without abnormal cord signal change. The lesion abuts the left vertebral artery.

No spinal malalignment. Patchy bone marrow present due to a combination of fatty and red marrow. C2/3 to C4/5 mild diffuse disc bulges, C7/T1 mild diffuse disc bulge.

Right-sided rounded homogenous enhancing acoustic neuroma of 7 mm at the level of the right internal auditory meatus. There is no intracanalicular component.

Fluid demonstrated within the right mastoid air cells suggestive of mastoiditis.

Conclusion

  1. Left C2/3 neural exit foramen peripheral nerve sheath tumor demonstrated related to C2 nerve. Mass effect upon spinal cord present at this level.
  2. Right-sided extra canalicular acoustic neuroma also identified. Therefore appearances suggest NF 2.

Case Discussion

This patient was known to have NF2.

NF2 should be renamed MISME syndrome. Schwannoma + vestibular neuroma = NF2 in the exam setting

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