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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, Sedivcová 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