Adolescent male with bilateral succinate dehydrogenase-deficient renal cell carcinoma in a horseshoe kidney managed successfully with staged bilateral robotic-assisted partial nephrectomies: A case report

Succinate dehydrogenase (SDH) deficient renal cell carcinoma (RCC) is a rare subset of familial RCC with only 59 cases reported. SDH deficiency is associated with hereditary paraganglioma/pheochromocytoma syndrome. Most of the cases are solitary tumors with only two reported cases of bilateral tumor. The identification of SDH deficient RCC is often the sentinel event of patient's syndromic diagnosis. We present a case of an adolescent male with bilateral tumors in a horseshoe kidney who was treated with staged robotic-assisted partial nephrectomies without complication. Both tumors were SDH negative on immunohistochemical staining.