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Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ®)

  • Posted: 11/01/2013
  • Updated: 08/01/2014

Table 2. Neoplasms in von Hippel-Lindau Syndrome: Mean Age at Diagnosis and Cumulative Risk in Patients Affected

Neoplasm Mean Age (Range) in y  Cumulative Risk (%)  
Adapted from Choyke et al.[4] and Lonser et al.[5]
Renal cell cancer 37 (16–67)24–45
Pheochromocytoma 30 (5–58)10–20
Pancreatic tumor or cyst 36 (5–70)35–70
Retinal hemangioblastoma 25 (1–67)25–60
Cerebellar hemangioblastoma 33 (9–78)44–72
Brainstem hemangioblastoma 32 (12–4610–25
Spinal cord hemangioblastoma 33 (12–66)13–50
Endolymphatic sac tumor 22 (12–50)10

References

  1. Choyke PL, Glenn GM, Walther MM, et al.: von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 194 (3): 629-42, 1995.  [PUBMED Abstract]

  2. Lonser RR, Glenn GM, Walther M, et al.: von Hippel-Lindau disease. Lancet 361 (9374): 2059-67, 2003.  [PUBMED Abstract]