. "Benn, Diana E." . . "000345131700002" . "Andresen, Per Arne" . "Chow, Chung W." . "Miettinen, Markku" . "Gill, Anthony J." . "Yong, Jim L." . "Succinate dehydrogenase (SDH)-deficient renal carcinoma has been accepted as a provisional entity in the 2013 International Society of Urological Pathology Vancouver Classification. To further define its morphologic and clinical features, we studied a multi-institutional cohort of 36 SDH-deficient renal carcinomas from 27 patients, including 21 previously unreported cases. We estimate that 0.05% to 0.2% of all renal carcinomas are SDH deficient. Mean patient age at presentation was 37 years (range, 14 to 76 y), with a slight male predominance (M: F = 1.7:1). Bilateral tumors were observed in 26% of patients. Thirty-four (94%) tumors demonstrated the previously reported morphology at least focally, which included: solid or focally cystic growth, uniform cytology with eosinophilic flocculent cytoplasm, intracytoplasmic vacuolations and inclusions, and round to oval low-grade nuclei. All 17 patients who underwent genetic testing for mutation in the SDH subunits demonstrated germline mutations (16 in SDHB and 1 in SDHC). Nine of 27 (33%) patients developed metastatic disease, 2 of them after prolonged follow-up (5.5 and 30 y). Seven of 10 patients (70%) with high-grade nuclei metastasized as did all 4 patients with coagulative necrosis. Two of 17 (12%) patients with low-grade nuclei metastasized, and both had unbiopsied contralateral tumors, which may have been the origin of the metastatic disease. In conclusion, SDH-deficient renal carcinoma is a rare and unique type of renal carcinoma, exhibiting stereotypical morphologic features in the great majority of cases and showing a strong relationship with SDH germline mutation. Although this tumor may undergo dedifferentiation and metastasize, sometimes after a prolonged delay, metastatic disease is rare in the absence of high-grade nuclear atypia or coagulative necrosis."@en . "Yakirevich, Evgeny" . "Watson, Nicole" . . "12" . "Tan, Puay Hoon" . "Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients" . "Sioson, Loretta" . "\u0160edivcov\u00E1, Monika" . "38" . "38"^^ . "Yilmaz, Asli" . "Succinate dehydrogenase (SDH)-deficient renal carcinoma has been accepted as a provisional entity in the 2013 International Society of Urological Pathology Vancouver Classification. To further define its morphologic and clinical features, we studied a multi-institutional cohort of 36 SDH-deficient renal carcinomas from 27 patients, including 21 previously unreported cases. We estimate that 0.05% to 0.2% of all renal carcinomas are SDH deficient. Mean patient age at presentation was 37 years (range, 14 to 76 y), with a slight male predominance (M: F = 1.7:1). Bilateral tumors were observed in 26% of patients. Thirty-four (94%) tumors demonstrated the previously reported morphology at least focally, which included: solid or focally cystic growth, uniform cytology with eosinophilic flocculent cytoplasm, intracytoplasmic vacuolations and inclusions, and round to oval low-grade nuclei. All 17 patients who underwent genetic testing for mutation in the SDH subunits demonstrated germline mutations (16 in SDHB and 1 in SDHC). Nine of 27 (33%) patients developed metastatic disease, 2 of them after prolonged follow-up (5.5 and 30 y). Seven of 10 patients (70%) with high-grade nuclei metastasized as did all 4 patients with coagulative necrosis. Two of 17 (12%) patients with low-grade nuclei metastasized, and both had unbiopsied contralateral tumors, which may have been the origin of the metastatic disease. In conclusion, SDH-deficient renal carcinoma is a rare and unique type of renal carcinoma, exhibiting stereotypical morphologic features in the great majority of cases and showing a strong relationship with SDH germline mutation. Although this tumor may undergo dedifferentiation and metastasize, sometimes after a prolonged delay, metastatic disease is rare in the absence of high-grade nuclear atypia or coagulative necrosis." . "11140" . "RIV/00216208:11140/14:10283809" . "I" . . "2"^^ . "Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients"@en . "Clarkson, Adele" . . "Yu, Darryl" . . "Trpkov, Kiril" . "Przybycin, Christopher" . . "5"^^ . "48499" . "Hes, Ond\u0159ej" . "RIV/00216208:11140/14:10283809!RIV15-MSM-11140___" . . . "renal carcinoma; succinate dehydrogenase; SDHA; SDHB"@en . "Magi-Galluzzi, Cristina" . "Vlatkovic, Ljiljana" . "Robinson, Bruce G." . . "Papathomas, Thomas" . "McKenney, Jesse K." . "Hills, Kirsten" . "Toon, Christopher W." . "Michal, Michal" . "Agaimy, Abbas" . . "Fleming, Stewart" . "Clifton-Bligh, Roderick J." . "Paik, Julie" . "Kedziora, Andrew" . "American Journal of Surgical Pathology" . "Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients" . . "Nicoll, Katherine D." . "Hartmann, Arndt" . "[2E682A614C95]" . "Chou, Angela" . "0147-5185" . "Corssmit, Eleonora P. M." . "US - Spojen\u00E9 st\u00E1ty americk\u00E9" . "van Leenders, Geert J. L. H." . . . "Sibony, Mathilde" . "Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients"@en . . "Niemeijer, Nicolasine D." . "Maclean, Fiona" . .