Succinate dehydrogenase deficient renal cell carcinoma (SDH-RCC) is a very rare but distinct renal neo-plasm, most often presenting at a young age and commonly associated with paragangliomas, pheochromocytomas and gastro-intestinal stromal tumours as a hereditary cancer syndrome. Although SDH-RCCs often have a relatively indolent disease course, higher nuclear grade, coagulative necrosis and sarcomatoid dedifferentiation may indicate aggressive disease. Radical surgery and (targeted) radiation therapy are valuable options in the treatment of these rare tumours. Genetic testing for germline SDH mutations is crucial. First-line relatives with germline SDH mutations should undergo periodical screening since early detection is paramount. The strong presence of the Warburg effect in SDH-related tumours make these the hallmark tumour for 18Fluorodeoxyglucose Positron Emission Tomography based screening and follow-up.
(BELG J MED ONCOL 2021;15(1):44-7)