1. Inherited Syndrome

2. 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

3. MRI

6. CT

7. Hemangioblastoma Uncommon brain tumor of vascular origin Cerebellum 83% Spinal cord 13% Benign Sporadic tumor Usually solitary Von-Hippel-Lindau disease Frequently multiple tumors

8. 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)

9. 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

10. Post op MRI

11. Post op CT