title: Endolymphatic sac carcinoma of the right petrous bone in Von Hippel-Lindau disease.
abstract: A large endolymphatic sac papillary adenocarcinoma in a patient with Von Hippel-Lindau (vHL) disease is reported. A 31-year-old man was treated for a left eye retinal angioma 10 years previously and had been blind in that eye since. He was admitted with progressive tinnitus, lower cranial nerve paresis and ataxia. Investigations revealed a vascular and bone-eroding petrous tumour. The entirely extradural tumour involved a large part of the petrous bone and extended into the cerebellopontine angle. The vascular tumour was radically resected using a presigmoid approach after feeding vessel embolisation. The literature on this rare clinical entity is briefly discussed.
authors: Department of Neurosurgery, King Edward VII Memorial Hospital, Seth G.S. Medical College, Parel, Mumbai 400012, India.
authors: D P
authors: DP
authors: Muzumdar
authors: A
authors: A
authors: Goel
authors: S
authors: S
authors: Fattepurkar
authors: N
authors: N
authors: Goel