Familial Renal Oncocytoma

Familial renal oncocytoma is the occurrence of oncocytomas in two or more individuals in the same family with no evidence of any other hereditary kidney cancer syndrome, i.e. no mutation in any gene known to predispose to RCC.

Signs and symptoms:

  • Familial renal oncocytoma has an increased risk of benign renal oncocytomas, which may be multifocal and bilateral.
  • More than one family member with oncocytomas
  • Absence of fibrofolliculomas and lung cysts.


  • The associated gene(s) have not yet been identified. It is very important to find an expert in familial renal cancers if you suspect you may have this condition.
  • The physical symptoms may mimic one of the known syndromes, especially BHD, but gene tests of all possible candidate genes do not show any of the known mutations. No genetic test is available.




Publication date: September 2012
Review date: September 2015