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