Succinate dehydrogenase-deficient renal cell carcinoma

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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 described in 2012.[1]
  • May be assocated with paraganglioma (as with SDH mutations).

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. ↑ 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. ↑ 2.0 2.1 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. ↑ 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.
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