Von Hippel-Lindau disease

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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%