Succinate dehydrogenase-deficient renal cell carcinoma
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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 recently described.
- May be assocated with paraganglioma (as with SDH mutations).
Microscopic
Features:[2]
- Oncocytic cells with flocculent cytoplasmic inclusions.
- Intratumoural mast cells.
- Solid architecture.
DDx:
- Renal oncocytoma.
- Chromophobe renal cell carcinoma, oncocytic variant.
IHC
Features:
- SDHB -ve (11 of 11 cases[2]).
- CD117 -ve.
See also
References
- ↑ 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 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.