Although metastatic disease seems to be rare in SDHC mutation carriers, it has in a few cases been reported.40, 55, 56, 57 Eight RCC and multiple GISTs have been reported in SDHC carriers.19, 58, 59 Two cases of pituitary adenoma have been described, although for LoH studies no tissue was available to prove pathogenicity.24 Here, SDHC is linked to renal cell carcinoma.