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| Description
| - 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.
- 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)
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| Title
| - Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients
- Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients (en)
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| skos:prefLabel
| - Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients
- Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients (en)
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| skos:notation
| - RIV/00216208:11140/14:10283809!RIV15-MSM-11140___
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| http://linked.open...avai/riv/aktivita
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| http://linked.open...avai/riv/aktivity
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| http://linked.open...iv/cisloPeriodika
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| http://linked.open...vai/riv/dodaniDat
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| http://linked.open...aciTvurceVysledku
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| http://linked.open.../riv/druhVysledku
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| http://linked.open...iv/duvernostUdaju
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| http://linked.open...titaPredkladatele
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| http://linked.open...dnocenehoVysledku
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| http://linked.open...ai/riv/idVysledku
| - RIV/00216208:11140/14:10283809
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| http://linked.open...riv/jazykVysledku
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| http://linked.open.../riv/klicovaSlova
| - renal carcinoma; succinate dehydrogenase; SDHA; SDHB (en)
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| http://linked.open.../riv/klicoveSlovo
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| http://linked.open...odStatuVydavatele
| - US - Spojené státy americké
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| http://linked.open...ontrolniKodProRIV
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| http://linked.open...i/riv/nazevZdroje
| - American Journal of Surgical Pathology
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| http://linked.open...in/vavai/riv/obor
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| http://linked.open...ichTvurcuVysledku
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| http://linked.open...cetTvurcuVysledku
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| http://linked.open...UplatneniVysledku
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| http://linked.open...v/svazekPeriodika
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| http://linked.open...iv/tvurceVysledku
| - Hes, Ondřej
- Michal, Michal
- Šedivcová, Monika
- Agaimy, Abbas
- Tan, Puay Hoon
- Hartmann, Arndt
- McKenney, Jesse K.
- Andresen, Per Arne
- Benn, Diana E.
- Chou, Angela
- Chow, Chung W.
- Clarkson, Adele
- Clifton-Bligh, Roderick J.
- Corssmit, Eleonora P. M.
- Fleming, Stewart
- Gill, Anthony J.
- Hills, Kirsten
- Kedziora, Andrew
- Maclean, Fiona
- Magi-Galluzzi, Cristina
- Miettinen, Markku
- Nicoll, Katherine D.
- Niemeijer, Nicolasine D.
- Paik, Julie
- Papathomas, Thomas
- Przybycin, Christopher
- Robinson, Bruce G.
- Sibony, Mathilde
- Sioson, Loretta
- Toon, Christopher W.
- Trpkov, Kiril
- Vlatkovic, Ljiljana
- Watson, Nicole
- Yakirevich, Evgeny
- Yilmaz, Asli
- Yong, Jim L.
- Yu, Darryl
- van Leenders, Geert J. L. H.
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| http://linked.open...ain/vavai/riv/wos
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| issn
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| number of pages
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| http://localhost/t...ganizacniJednotka
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