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Transcript 
Inherited Syndrome
History
 55 year old female presents with headaches, slurred speech
and changes in short-term memory
 Pt also complained of gait abnormalities
 H/o renal cell carcinoma
MRI
MRI
MRI
CT
Hemangioblastoma
 Uncommon brain tumor of vascular origin
 Cerebellum 83%
 Spinal cord 13%
 Benign
 Sporadic tumor
 Usually solitary
 Von-Hippel-Lindau disease
 Frequently multiple tumors
Von-Hippel-Lindau Disease
 Autosomal Dominant syndrome
 Manifestations include a variety of benign and malignant tumors
 Hemangioblastomas
 Clear cell renal cell carcinomas
 Pheochromocytomas
 Endolymphatic sac tumors (middle ear)
 Serous cystadenomas and neuroendocrine tumors (pancreas)
 Papillary cystadenomas (epididymis and broad ligament)
Surveillance Protocol
 Annual Exam that includes:
 Plasma catecholamines and abdominal CT w/contrast
 Pheochromocytoma
 Retinal exam with pupil dilation
 Retinal angiomas
 MRI of the brain and entire spine with gadolinium
 Hemangioblastoma
 MRI or CT of the abdomen
 Renal cell carcinoma
Post op MRI
Post op CT
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