Von Hippel-Lindau disease
Associations with von Hippel-Lindau disease
| Common Lesions | Frequency in VHL | |
|---|---|---|
| Hemangioblastomas | - Cerebellum (solid or cystic) | 80% |
| - Retina | 41–59% | |
| - Brainstem | 10–25% | |
| - Spinal cord | 10–50% | |
| Pancreatic tumors or cysts | 22–80% | |
| Renal clear cell carcinoma & cysts | 14–60% | |
| Polycythemia | 9–20% of intracranial hemangioblastomas | |
| Rare Lesions (pertinent to nervous system) | ||
| Supratentorial hemangioblastoma | 3–6% | |
| Cystadenomas of the broad ligament | 10% of females | |
| Papillary cystadenomas of the epididymis | 25–60% of males | |
| Endolymphatic sac tumors | 10–15% | |
| Adrenal medullary pheochromocytoma (tends to be bilateral) | 7–24% | |