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

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**SDHA has been described.<ref name=pmid25034258 >{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Eble | first2 = JN. | last3 = Amin | first3 = MB. | last4 = Gupta | first4 = NS. | last5 = Smith | first5 = SC. | last6 = Sholl | first6 = LM. | last7 = Montironi | first7 = R. | last8 = Hirsch | first8 = MS. | last9 = Hornick | first9 = JL. | title = Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma. | journal = Mod Pathol | volume = 28 | issue = 1 | pages = 80-94 | month = Jan | year = 2015 | doi = 10.1038/modpathol.2014.86 | PMID = 25034258 }}</ref>
**SDHA has been described.<ref name=pmid25034258 >{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Eble | first2 = JN. | last3 = Amin | first3 = MB. | last4 = Gupta | first4 = NS. | last5 = Smith | first5 = SC. | last6 = Sholl | first6 = LM. | last7 = Montironi | first7 = R. | last8 = Hirsch | first8 = MS. | last9 = Hornick | first9 = JL. | title = Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma. | journal = Mod Pathol | volume = 28 | issue = 1 | pages = 80-94 | month = Jan | year = 2015 | doi = 10.1038/modpathol.2014.86 | PMID = 25034258 }}</ref>
**SDHC has been described.<ref name=pmid23083876/>
**SDHC has been described.<ref name=pmid23083876/>
*Usually good prognosis.


==Gross==
==Gross==

Revision as of 01:27, 26 March 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]
    • SDHA has been described.[2]
    • SDHC has been described.[3]
  • Usually good prognosis.

Gross

  • Commonly cystic.

Microscopic

Features:[2]

  • Eosinophilic cells with clear (flocculent) cytoplasmic inclusions. ‡
  • Round nuclei with mildly granular chromatin pattern.
  • Intratumoural mast cells.
  • Solid architecture.

Notes:

DDx:

Images

IHC

Features:

  • SDHB -ve (11 of 11 cases[2]).
    • Normal tubules +ve.
    • May be (pseudo)negative in clear cells (as few mitochondria).
  • CD117 -ve - important.

Others:

  • PAX8 +ve.
  • CK7 -ve (1/35).

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 2.4 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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