Difference between revisions of "Succinate dehydrogenase-deficient renal cell carcinoma"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = SDH-deficient_renal_cell_carcinoma_-_high_mag.jpg | ||
| Width = | | Width = | ||
| Caption = | | Caption = SDH-deficient renal cell carcinoma. [[H&E stain]]. | ||
| Synonyms = | | Synonyms = | ||
| Micro = eosinophilic cells with clear (flocculent) cytoplasmic inclusions, round nuclei with mildly granular chromatin pattern, intratumoural [[mast cell]]s, solid architecture | | Micro = eosinophilic cells with clear (flocculent) cytoplasmic inclusions, round nuclei with mildly granular chromatin pattern, intratumoural [[mast cell]]s, solid architecture | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[renal oncocytoma]], [[chromophobe renal cell carcinoma]], [[clear cell renal cell carcinoma]], ther [[renal tumours with eosinophilic cytoplasm]] | | LMDDx = [[renal oncocytoma]], [[chromophobe renal cell carcinoma]], [[clear cell renal cell carcinoma]], ther [[renal tumours with eosinophilic cytoplasm]] | ||
| Stains = SDHB -ve, CK7 -ve | | Stains = SDHB -ve, CK7 -ve, CD117 -ve, PAX8 +ve | ||
| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Line 18: | Line 18: | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Line 64: | Line 64: | ||
===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> | *[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> | |||
==IHC== | ==IHC== | ||
Revision as of 05:18, 3 May 2015
| Succinate dehydrogenase-deficient renal cell carcinoma | |
|---|---|
| Diagnosis in short | |
 SDH-deficient renal cell carcinoma. H&E stain. | |
|
| |
| LM | eosinophilic cells with clear (flocculent) cytoplasmic inclusions, round nuclei with mildly granular chromatin pattern, intratumoural mast cells, solid architecture |
| LM DDx | renal oncocytoma, chromophobe renal cell carcinoma, clear cell renal cell carcinoma, ther renal tumours with eosinophilic cytoplasm |
| Stains | SDHB -ve, CK7 -ve, CD117 -ve, PAX8 +ve |
| Gross | mass lesion, commonly cystic |
| Site | kidney - see kidney tumours |
|
| |
| Prevalence | extremely rare |
| Prognosis | usually good prognosis (limited data) |
| Clin. DDx | other kidney tumours |
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]
- 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:
- ‡ Occasional cells may look like signet ring cells.
- Typically low ISUP nucleolar grade.
- May be sarcomatoid.
DDx:
- Renal oncocytoma - granular cytoplasm.
- Chromophobe renal cell carcinoma.
- Clear cell renal cell carcinoma.
- Other renal tumours with eosinophilic cytoplasm.
Images
www:
- SDHB-RCC - poor quality histology image (nih.gov).[3]
- SDHC-RCC (nih.gov).[3]
- SDH-deficient RCC (nature.com).[2]
Case 1
SDH-RCC low mag.
SDH-RCC intermed. mag.
SDH-RCC intermed. mag.
SDH-RCC high mag.
SDH-RCC very high mag.
Case 2
SDH-RCC intermed. mag.
SDH-RCC high mag.
SDH-RCC very high mag.
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
- ↑ 1.0 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.
- ↑ 2.0 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 - ↑ 3.0 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.