Succinate dehydrogenase-deficient renal cell carcinoma
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| Succinate dehydrogenase-deficient renal cell carcinoma | |
|---|---|
| Diagnosis in short | |
|
| |
| 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 |
| 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
- SDHB-RCC - poor quality histology image (nih.gov).[3]
- SDHC-RCC (nih.gov).[3]
- SDH-deficient RCC (nature.com).[2]
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.