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Succinate dehydrogenase-deficient renal cell carcinoma (view source)

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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>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = SDH-deficient_renal_cell_carcinoma_-_high_mag.jpg
| Width      =
| Caption    = SDH-deficient renal cell carcinoma (left of image). [[H&E stain]].
| Synonyms  =
| Micro      = eosinophilic cells with clear (flocculent) cytoplasmic inclusions, round nuclei with mildly granular chromatin pattern, intratumoural [[mast cell]]s, solid architecture
| Subtypes  =
| LMDDx      = [[renal oncocytoma]], [[chromophobe renal cell carcinoma]], [[clear cell renal cell carcinoma]], other [[renal tumours with eosinophilic cytoplasm]]
| Stains    =  
| IHC        = SDHB -ve, CK7 -ve, CD117 -ve, PAX8 +ve, CA9 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      = mass lesion, commonly cystic
| Grossing  = [[partial nephrectomy grossing]], [[total nephrectomy for tumour grossing]]
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      =
| Syndromes  = familial pheochromocytoma and renal cell carcinoma syndrome
| Clinicalhx =  
| Signs      =
| Symptoms  =
| Prevalence = extremely rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = usually good prognosis (limited data)
| Other      =
| ClinDDx    = other [[kidney tumours]]
| Tx        =
}}
'''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 in the 2016 World Health Organization classification of renal neoplasia.


==General==
==General==
*Extremely rare - only approximately 10 cases described as of 2012.<ref name=pmid24025519/>
*Extremely rare - estimated to represent up to 0.2% of [[renal cell carcinoma]]s.<ref name=pmid25025441>{{Cite journal  | last1 = Gill | first1 = AJ. | last2 = Hes | first2 = O. | last3 = Papathomas | first3 = T. | last4 = Šedivcová | first4 = M. | last5 = Tan | first5 = PH. | last6 = Agaimy | first6 = A. | last7 = Andresen | first7 = PA. | last8 = Kedziora | first8 = A. | last9 = Clarkson | first9 = A. | title = Succinate dehydrogenase (SDH)-deficient renal carcinoma: a morphologically distinct entity: a clinicopathologic series of 36 tumors from 27 patients. | journal = Am J Surg Pathol | volume = 38 | issue = 12 | pages = 1588-602 | month = Dec | year = 2014 | doi = 10.1097/PAS.0000000000000292 | PMID = 25025441 }}</ref>
*May be assocated with [[paraganglioma]] (as with several SDH mutations).
*May be assocated with [[paraganglioma]] and familial, as with several SDH mutations.<ref name=pmid25800244>{{Cite journal  | last1 = Hernandez | first1 = KG. | last2 = Ezzat | first2 = S. | last3 = Morel | first3 = CF. | last4 = Swallow | first4 = C. | last5 = Otremba | first5 = M. | last6 = Dickson | first6 = BC. | last7 = Asa | first7 = SL. | last8 = Mete | first8 = O. | title = Familial pheochromocytoma and renal cell carcinoma syndrome: TMEM127 as a novel candidate gene for the association. | journal = Virchows Arch | volume =  | issue =  | pages =  | month = Mar | year = 2015 | doi = 10.1007/s00428-015-1755-2 | PMID = 25800244 }}</ref>
*Primarily described with a ''SDHB mutation''.<ref name=pmid25034258/>
*Primarily described with a ''SDHB mutation''.<ref name=pmid25034258/>
**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/>
<!-- **SDHD has been described.{{fact}} -->
*Usually good prognosis.
==Gross==
*Mass lesion - commonly cystic.


==Microscopic==
==Microscopic==
Features:<ref name=pmid25034258/>
Features:<ref name=pmid25034258/>
*Oncocytic cells with flocculent cytoplasmic inclusions.
*Eosinophilic cells with clear (flocculent) cytoplasmic inclusions. ‡
*Intratumoural [[mast cell]]s.
*Round nuclei with mildly granular chromatin pattern.
*Solid architecture.
*Intratumoural [[mast cell]]s - usu. not prominent.
*Solid architecture - typical.
 
Notes:
*‡ Occasional cells may look like [[signet ring cells]].
*Typically low [[ISUP nucleolar grade]].
**May be [[renal cell carcinoma with sarcomatoid differentiation|sarcomatoid]].


DDx:
DDx:
*[[Renal oncocytoma]].
*[[Renal oncocytoma]] - granular cytoplasm.
*[[Chromophobe renal cell carcinoma]].
*[[Chromophobe renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]].
*Other [[renal tumours with eosinophilic cytoplasm]].


===Images===
===Images===
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856891/figure/F3/ SDHB-RCC - poor quality histology image (nih.gov)].<ref name=pmid23083876/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856891/figure/F3/ SDHB-RCC - poor quality histology image (nih.gov)].<ref name=pmid23083876/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856891/figure/F6/ SDHC-RCC (nih.gov)].<ref name=pmid23083876>{{Cite journal  | last1 = Ricketts | first1 = CJ. | last2 = Shuch | first2 = B. | last3 = Vocke | first3 = CD. | last4 = Metwalli | first4 = AR. | last5 = Bratslavsky | first5 = G. | last6 = Middelton | first6 = L. | last7 = Yang | first7 = Y. | last8 = Wei | first8 = MH. | last9 = Pautler | first9 = SE. | title = Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer. | journal = J Urol | volume = 188 | issue = 6 | pages = 2063-71 | month = Dec | year = 2012 | doi = 10.1016/j.juro.2012.08.030 | PMID = 23083876 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856891/figure/F6/ SDHC-RCC (nih.gov)].<ref name=pmid23083876>{{Cite journal  | last1 = Ricketts | first1 = CJ. | last2 = Shuch | first2 = B. | last3 = Vocke | first3 = CD. | last4 = Metwalli | first4 = AR. | last5 = Bratslavsky | first5 = G. | last6 = Middelton | first6 = L. | last7 = Yang | first7 = Y. | last8 = Wei | first8 = MH. | last9 = Pautler | first9 = SE. | title = Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer. | journal = J Urol | volume = 188 | issue = 6 | pages = 2063-71 | month = Dec | year = 2012 | doi = 10.1016/j.juro.2012.08.030 | PMID = 23083876 }}</ref>
*[http://www.nature.com/modpathol/journal/v28/n1/fig_tab/modpathol201486f2.html SDH-deficient RCC (nature.com)].<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>
====Case 1====
<gallery>
Image: SDH-deficient renal cell carcinoma - low mag.jpg | SDH-RCC low mag.
Image: SDH-deficient renal cell carcinoma - intermed mag.jpg | SDH-RCC intermed. mag.
Image: SDH-deficient renal cell carcinoma - alt - intermed mag.jpg | SDH-RCC intermed. mag.
Image: SDH-deficient renal cell carcinoma - high mag.jpg | SDH-RCC high mag.
Image: SDH-deficient renal cell carcinoma - very high mag.jpg | SDH-RCC very high mag.
</gallery>
====Case 2====
<gallery>
Image: SDH-deficient renal cell carcinoma -2- intermed mag.jpg | SDH-RCC intermed. mag.
Image: SDH-deficient renal cell carcinoma -2- high mag.jpg | SDH-RCC high mag.
Image: SDH-deficient renal cell carcinoma -2- very high mag.jpg | SDH-RCC very high mag.
</gallery>
====Case 3====
Pictures demonstrates the variability between different regions of the tumour:
<gallery>
Image: SDH-RCC - 3 - characteristic - low mag.jpg | SDH-RCC - low mag.
Image: SDH-RCC - 3 - characteristic - intermed mag.jpg | SDH-RCC - intermed. mag.
Image: SDH-RCC - 3 - characteristic - high mag.jpg | SDH-RCC - high mag.
Image: SDH-RCC - 3 - myxoid change - low mag.jpg | SDH-RCC - low mag.
Image: SDH-RCC - 3 - myxoid change - intermed mag.jpg | SDH-RCC - intermed. mag.
Image: SDH-RCC - 3 - myxoid change - high mag.jpg | SDH-RCC - high mag.
Image: SDH-RCC - 3 - pseudo-oncocytes - low mag.jpg | SDH-RCC - low mag.
Image: SDH-RCC - 3 - pseudo-oncocytes - intermed mag.jpg | SDH-RCC - intermed. mag.
Image: SDH-RCC - 3 - pseudo-oncocytes - high mag.jpg | SDH-RCC - high mag.
</gallery>


==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/>).
*CD117 -ve.
**Normal tubules +ve.
**May be (pseudo)negative in clear cells (as few mitochondria).
*CD117 -ve - '''important'''.
*CA9 -ve.
 
Others:
*PAX8 +ve.
*EMA +ve (10/10<ref name=pmid25034258/>).
*CK7 -ve (1 +ve/35<ref name=pmid25025441/>).
*AE1/AE3 -ve/+ve (4 +ve/10<ref name=pmid25034258/>).
*CK20 -ve.
*CD10 usually focal (most common) or -ve.<ref name=pmid25025441/>


==See also==
==See also==
*[[Kidney tumours]].
*[[Kidney tumours]].
*[[Succinate dehydrogenase]].


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
==External links==
*[http://www.cancerdxpathology.org.au/virtual-sdh-rc.html Succinate dehydrogenase-deficient renal carcinoma (cancerdxpathology.org.au)] - virtual slides from Gill.


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