Endolymphatic sac tumor

Case contributed by Bruno Di Muzio
Diagnosis certain

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: 35 years
Gender: Female
ct
This study is a stack
Axial bone
window
This study is a stack
Axial C+ portal
venous phase
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Info

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
This study is a stack
Axial
FLAIR
This study is a stack
3D Time-of-flight
MRA
This study is a stack
Sagittal
MRA
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Info

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

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

mri
Axial
T2
Coronal
T2
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Info

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 tumor, 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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