Difference between revisions of "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 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''' 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>
==General==
*Extremely rare - only recently described.
*May be assocated with [[paraganglioma]] (as with SDH mutations).
==Microscopic==
Features:<ref name=pmid25034258/>
*Oncocytic cells with flocculent cytoplasmic inclusions.
*Intratumoural [[mast cell]]s.
*Solid architecture.
DDx:
*[[Renal oncocytoma]].
*[[Chromophobe renal cell carcinoma]], oncocytic variant.


==IHC==
==IHC==
Features:
Features:
*SDHB -ve (11 of 11 cases<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 =  | issue =  | pages =  | month = Jul | year = 2014 | doi = 10.1038/modpathol.2014.86 | PMID = 25034258 }}</ref>).
*SDHB -ve (11 of 11 cases<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 =  | issue =  | pages =  | month = Jul | year = 2014 | doi = 10.1038/modpathol.2014.86 | PMID = 25034258 }}</ref>).
*CD117 -ve.


==See also==
==See also==
Line 9: Line 24:


==References==
==References==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Kidney tumours]]
[[Category:Kidney tumours]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Revision as of 05:13, 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 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:

IHC

Features:

  • SDHB -ve (11 of 11 cases[2]).
  • CD117 -ve.

See also

References

  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.
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