Table 1 Cancer incidence and types of tumors in VHL disease[6]

CNS

  Hemangioblastomas of the retina

25-60%

  Endolymphatic Sac Tumors

10%

  Hemangioblastomas of

  Cerebellum

44-72%

  Brain stem

10-25%

  Spinal cord

13-50%

  Nerve roots L/S

< 1%

  Supratentorial region

< 1%

Organs

  Renal cel carcinoma

25-60%

  Pheochromocytoma

10-20%

  Pancreatic tumors

35-70%

  Epididymal cystadenomas

25-60%