Difference between revisions of "Succinate dehydrogenase-deficient renal cell carcinoma"
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==General== | ==General== | ||
*Extremely rare - only approximately 10 described | *Extremely rare - only approximately 10 cases described as of 2012.<ref name=pmid24025519/> | ||
*May be assocated with [[paraganglioma]] (as with several SDH mutations). | *May be assocated with [[paraganglioma]] (as with several SDH mutations). | ||
*Primarily described with a ''SDHB mutation''.<ref name=pmid25034258/> | *Primarily described with a ''SDHB mutation''.<ref name=pmid25034258/> | ||
Revision as of 05:37, 27 October 2014
Succinate dehydrogenase-deficient renal cell carcinoma, also succinate dehydrogenase renal cell carcinoma (abbreviated SDH-RCC), is a very rare type of renal cell carcinoma that is grouped in the emerging entities of the Vancouver modification of the 2004 WHO classification of renal neoplasia.[1]
General
- Extremely rare - only approximately 10 cases described as of 2012.[1]
- May be assocated with paraganglioma (as with several SDH mutations).
- Primarily described with a SDHB mutation.[2]
- SDHC has been described.[3]
Microscopic
Features:[2]
- Oncocytic cells with flocculent cytoplasmic inclusions.
- Intratumoural mast cells.
- Solid architecture.
DDx:
Images
IHC
Features:
- SDHB -ve (11 of 11 cases[2]).
- CD117 -ve.
See also
References
- ↑ 1.0 1.1 Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
- ↑ 2.0 2.1 2.2 Williamson, SR.; Eble, JN.; Amin, MB.; Gupta, NS.; Smith, SC.; Sholl, LM.; Montironi, R.; Hirsch, MS. et al. (Jul 2014). "Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma.". Mod Pathol. doi:10.1038/modpathol.2014.86. PMID 25034258.
- ↑ 3.0 3.1 3.2 Ricketts, CJ.; Shuch, B.; Vocke, CD.; Metwalli, AR.; Bratslavsky, G.; Middelton, L.; Yang, Y.; Wei, MH. et al. (Dec 2012). "Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer.". J Urol 188 (6): 2063-71. doi:10.1016/j.juro.2012.08.030. PMID 23083876.