Difference between revisions of "Succinate dehydrogenase-deficient renal cell carcinoma"

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==General==
==General==
*Extremely rare - only recently described.
*Extremely rare - only approximately 10 described in 2012.<ref name=pmid24025519/>
*May be assocated with [[paraganglioma]] (as with SDH mutations).
*May be assocated with [[paraganglioma]] (as with SDH mutations).



Revision as of 05:20, 27 October 2014

Succinate dehydrogenase-deficient renal cell carcinoma 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:

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.