Endolymphatic sac tumor

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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