A 30 year old male presented with headache.

1. What is the diagnosis?

2. This diagnosis is found in what syndrome?

3. What is classic for the diagnosis?

Correct answer:

1. What is the diagnosis?

Endolymphatic Sac Tumor

2. This diagnosis is found in what syndrome?

 Von Hippel Lindau

3. What is classic for the diagnosis?

The location

Endolymphatic Sac Tumor

Rare locally invasive tumor of the endolymph sac. It doesn’t metastasize, but does locally invade and cause hearing loss.

- The location is very typical (the region of the endolymph sac).

- The boney erosion pattern is sometimes called “moth-eaten”

- Highly associated with VHL (about 15% of VHLers have them)

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