Endolymphatic sac carcinoma of the right petrous bone in Von Hippel-Lindau disease.
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.
Muzumdar
D P
DP
Department of Neurosurgery, King Edward VII Memorial Hospital, Seth G.S. Medical College, Parel, Mumbai 400012, India.
Goel
A
A
Fattepurkar
S
S
Goel
N
N