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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== | ||
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==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Kidney tumours]] | [[Category:Kidney tumours]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] |
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