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

From: Acute myeloid leukemia in a 38-year-old hemodialyzed patient with von Hippel-Lindau disease

Type of cancer Cancer incidence
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%