Endolymphatic sac tumour

Case contributed by Dr Bruno Di Muzio

Presentation

Patient with von Hippel-Lindau syndrome presents with an expansile lesion in jugular foramen and retro labyrinthine portion of the right temporal bone.

Patient Data

Age: 33
Gender: Female
CT

CT Brain

Mass that erodes and invades the posterior wall of petrous part of the right temporal bone (retro labyrinthine portion of the temporal bone), involving part of the jugular foramen.

MRI

MRI Brain (selected images)

Mass eroding and invading the retro labyrinthine portion of the right temporal bone. 

Extra-axial lesion without promoting thrombosis of the venous sinuses.

MRI

MRI study of abdome that shows another vHL common feature: multiple renal cysts

Bilateral and multiple renal cysts.

Case Discussion

Patient with von Hippel-Lindau syndrome (vHL) and had previously resected a left cerebellar hemangioblastoma (signs of cranioplasty on CT).

These studies show a mass in retro labyrinthine portion of the right temporal bone, involving part of jugular foramen, that was confirmed as an endolymphatic sac tumour, which occurs in about 16% of patients with von Hippel-Lindau syndrome.

This patient also has an MRI study of the abdomen showing another vHL feature: multiple renal cysts.

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Case information

rID: 27278
Case created: 26th Jan 2014
Last edited: 2nd Nov 2016
Inclusion in quiz mode: Included

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