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
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.
Mass eroding and invading the retro labyrinthine portion of the right temporal bone.
Extra-axial lesion without promoting thrombosis of the venous sinuses.
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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