In rare cases, characteristic histopathological features may suggest an underlying inherited disorder (e.g. SDH-deficient RCC, hybrid chromophobe-oncocytic and BHD syndrome), but, in general, in the absence of family history or multicentric disease, age at diagnosis seems to be the most practical approach (with 70% general consensus) (38) for stratifying genetic testing. The gene discussed is SDHB; the disease is Birt-Hogg-Dubé syndrome.