Although approximately 14 confirmed genes are associated with pheochromocytoma and paraganglioma susceptibility, the genes investigated in the present case – SDHB, SDHC, SDHD, VHL and MAX – have been the main reported genes associated with malignant PCC reported so far, according to the Endocrine Society Clinical Practice Guideline (1). This evidence concerns the gene VHL and adrenal gland pheochromocytoma.