People who have family members with kidney cancer, especially a sibling, are at increased risk. This can be due to genes that pass down from parent to child. Inherited gene mutations cause only 3-5 % of kidney cancer. Signs that your kidney cancer might be hereditary include:

  • You have more than one tumour in your kidney (multifocal tumours)
  • You have tumours in both kidneys (bilateral tumours)
  • You have a rarer form of kidney cancer (a non-clear cell renal cell carcinoma)
  • Other members of your family have had kidney cancer
  • You had your first kidney tumour before you were 50 years old.

If there is a history of kidney cancer in your family, it is important that you tell your doctor so you can be tested. If the test shows that you do have a hereditary type of kidney cancer, other members of your family can be tested so that any sign of cancer could be treated early when it is most curable.

Types of hereditary kidney cancer

There are several different types of hereditary kidney cancer. In the future we may have more information about new genes that cause kidney cancer.

  • Von Hippel–Lindau disease (VHL): This is caused by a mutation in the VHL gene and usually causes clear-cell renal cell carcinoma.
  • Hereditary leiomyomatosis and renal cell cancer (HLRCC): This is caused by a mutation in the FH gene and usually causes papillary type 2 renal cell carcinoma.
  • Hereditary papillary renal cell carcinoma (HPRC): This is caused by a mutation in the MET gene and usually causes papillary type 1 renal cell carcinoma.
  • Birt–Hogg–Dubé (BHD): This is caused by a mutation in the FLCN gene and usually causes chromophobe renal cell carcinoma or oncocytoma.
  • Renal cell carcinoma with hereditary paraganglioma and phaeochromocytoma: This is caused by mutations in the SDHB or SDHD gene.
  • Chromosome 3 translocation familial renal cell carcinoma: This is caused by a chromosome 3 translocation. A chromosome translocation is when part of a chromosome breaks off and attaches to a different chromosome.
  • Tuberous sclerosis complex (TSC): This is caused by a mutation in the TSC1 or TSC2 gene and usually
    causes a type of kidney cancer called angiomyolipoma. These tumours are benign, but they have a
    large number of blood vessels which can burst and lead to life-threatening internal bleeding if not
    treated.

Kidney cancer in children

In rare cases, children can get kidney cancer, but they usually develop different types of kidney cancer than adults. The most common types of childhood kidney cancer are Wilms tumour and nephroblastoma. However, there have been rare cases of children with renal cell carcinoma or adults with Wilms tumour.

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