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SDH-associated renal cell carcinoma

Wednesday 17 December 2014

SDH-related renal cell carcinoma; Succinate dehydrogenase (SDH)-deficient renal carcinoma

Succinate dehydrogenase (SDH)-deficient renal carcinoma has been accepted as a provisional entity in the 2013 International Society of Urological Pathology Vancouver Classification.

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. (25025441)

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.(25025441)

In a study, 94% of 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,
- round to oval low-grade nuclei.

Open references

- Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity: A Clinicopathologic Series of 36 Tumors From 27 Patients. Gill AJ, Hes O, Papathomas T, Sedivcov√° M, Tan PH, Agaimy A, Andresen PA, Kedziora A, Clarkson A, Toon CW, Sioson L, Watson N, Chou A, Paik J, Clifton-Bligh RJ, Robinson BG, Benn DE, Hills K, Maclean F, Niemeijer ND, Vlatkovic L, Hartmann A, Corssmit EP, van Leenders GJ, Przybycin C, McKenney JK, Magi-Galluzzi C, Yilmaz A, Yu D, Nicoll KD, Yong JL, Sibony M, Yakirevich E, Fleming S, Chow CW, Miettinen M, Michal M, Trpkov K. Am J Surg Pathol. 2014 Dec;38(12):1588-602. doi : 10.1097/PAS.0000000000000292 PMID: 25025441