A 55-year-old woman presents with slowly progressive hearing loss and tinnitus. MRI shows bilateral masses at the cerebellopontine angle extending into the internal acoustic meatus. Histology shows Antoni A areas with Verocay bodies. What is the most likely underlying diagnosis?
- A Von Hippel-Lindau disease
- B Neurofibromatosis type 2 ✓
- C Neurofibromatosis type 1
- D Tuberous sclerosis
Correct answer: B. Neurofibromatosis type 2
Explanation
Bilateral vestibular schwannomas are pathognomonic for NF2, caused by mutation of the NF2 gene on chromosome 22 encoding merlin. Schwannomas show biphasic histology with cellular Antoni A areas containing palisaded nuclei around acellular zones called Verocay bodies. NF1 is associated with neurofibromas rather than schwannomas, and its gene product is neurofibromin, which kills options A and C.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.