Clinical manifestations of von Hippel-Lindau disease

	Ages at diagnosis (years)	Most common ages at diagnosis (years)	Frequency in patients
CNS
Retinal hemangioblastomas	0 to 68	12 to 25	25 to 60%
Endolymphatic sac tumors	12 to 46	24 to 35	10 to 25%
Cerebellar hemangioblastomas	9 to 78	18 to 25	44 to 72%
Brainstem hemangioblastomas	12 to 36	24 to 35	10 to 25%
Spinal cord hemangioblastomas	12 to 66	24 to 35	13 to 50%
Viscera
Renal cell carcinoma or cysts	16 to 67	25 to 50	25 to 60%
Pheochromocytomas^*	4 to 58	12 to 25	10 to 20%^¶
Pancreatic tumor or cyst	5 to 70	24 to 35	35 to 70%
Epididymal cystadenomas	17 to 43	14 to 40	25 to 60% of males
Adnexal papillary cystadenoma of mesonephric origin (broad ligament cystadenoma)	16 to 64	16 to 46	Estimated 10% of females

CNS: central nervous system.

* Includes the 20% of lesions that occur outside the adrenal gland, also called paragangliomas.

¶ Frequency of pheochromocytoma varies widely depending on genotype.

Data from: Lonser RR, Glenn GM, Walther M, et al. von Hippel-Lindau disease. Lancet 2003; 361:2059.

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