Von Hippel-Lindau Disorder

•Autosomal dominant disorder (Chroosome 3) with 1/35000-1/40000 incidence in America
•Most attacked in 2nd to 3rd decades
•M:F is about 1:1
Diagnostic Criteria
•> 1 hemangioblastoma of CNS
•1 hemangioblastoma + visceral manifestation
•1 manifestation + family history

Subclassification by NIH

•Type I: Renal + pancreatic cysts, high risk for RCC, no pheochromocytoma
•Type IIA: Pheochromocytoma, pancreatic islet cell tumor
•Type IIB: Pheochromocytoma + renal + pancreatic disease

Angiomatous lesions

- Retinal hemangioblastomas -> Von Hippel tumor
- CNS hemangioblastomas -> Lindau tumor
- Endolymphatic sac tumors
- Renal cell carcinoma - Pancreatic cysts and tumors
- Pheochromocytomas
- Epididymal cystadenomas