Difference between revisions of "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 classification|Vancouver modification of the 2004 WHO classification of renal neoplasia]]''.<ref name=pmid24025519>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref>
'''Succinate dehydrogenase-deficient renal cell carcinoma''', also '''succinate dehydrogenase renal cell carcinoma''' (abbreviated '''SDH-RCC'''), is a very rare type of [[renal cell carcinoma]].
 
It is grouped in the ''emerging entities'' of the ''[[Vancouver classification|Vancouver modification of the 2004 WHO classification of renal neoplasia]]''.<ref name=pmid24025519>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref>


==General==
==General==

Revision as of 21:43, 4 February 2015

Succinate dehydrogenase-deficient renal cell carcinoma, also succinate dehydrogenase renal cell carcinoma (abbreviated SDH-RCC), is a very rare type of renal cell carcinoma.

It 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 - important.

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 2.2 2.3 Williamson, SR.; Eble, JN.; Amin, MB.; Gupta, NS.; Smith, SC.; Sholl, LM.; Montironi, R.; Hirsch, MS. et al. (Jan 2015). "Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma.". Mod Pathol 28 (1): 80-94. doi:10.1038/modpathol.2014.86. PMID 25034258. Cite error: Invalid <ref> tag; name "pmid25034258" defined multiple times with different content
  3. ↑ 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.
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