Table 2.

Lifetime risks of VHL-associated tumors

TumoraRiskYoungest/mean age of diagnosis (years)
CNS hemangioblastoma60%–80%9/30
 Cerebellar44%–72%9/31
 Brainstem10%–25%12/32
 Spinal13%–50%8/33
Retinal angioma/hemangioblastoma25%–60%0/25
Renal25%–75%12/39
 Cyst42%12/37
 RCCb17%–70%13/44
PHEOc10%–25%2/27
ELSTd10%–15%6/22
Pancreatic35%–75%5/36
 Cyst21%5/33
 NETe10%–17%16/35
Papillary cystadenoma
 Epididymis25%–60%17/24
 Broad ligamentf10%16/unknown (16–46)
  • aReferences 6, 23, and 24.

  • bReferences 7, 11, 31, and 70.

  • cReference 10.

  • dReference 8.

  • eReference 9.

  • fReference 6.