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

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

Table 3. Genotype-Phenotype Classification of Families With von Hippel-Lindau Syndromea

Type  Clinical Characteristics 
1Retinal hemangioblastomas
CNS hemangioblastomas
Renal cell cancers
Pancreatic neoplasms and cysts
2APheochromocytomas
Retinal hemangioblastomas
CNS hemangioblastomas
2BPheochromocytomas
Retinal hemangioblastomas
CNS hemangioblastomas
Renal cell cancers
Pancreatic neoplasms and cysts
2CPheochromocytomas only

CNS = central nervous system.
aAdapted from Lonser et al.[5]

References

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