Difference between revisions of "ELOC-mutated renal cell carcinoma"
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''' | '''ELOC-mutated renal cell carcinoma''' is a type of [[renal cell carcinoma]] that is morphologically similar to [[clear cell renal cell carcinoma]] and [[clear cell tubulopapillary renal cell carcinoma]].<ref name=pmid25676555>{{Cite journal | last1 = Hakimi | first1 = AA. | last2 = Tickoo | first2 = SK. | last3 = Jacobsen | first3 = A. | last4 = Sarungbam | first4 = J. | last5 = Sfakianos | first5 = JP. | last6 = Sato | first6 = Y. | last7 = Morikawa | first7 = T. | last8 = Kume | first8 = H. | last9 = Fukayama | first9 = M. | title = TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype. | journal = Mod Pathol | volume = 28 | issue = 6 | pages = 845-53 | month = Jun | year = 2015 | doi = 10.1038/modpathol.2015.6 | PMID = 25676555 }}</ref> It was formally recognized in the WHO 2022 classification of renal neoplasia.{{fact}} | ||
It should '''not''' be confused with [[TFEB renal cell carcinoma]], also known as ''renal tumour with t(6;11) translocation''. | It was previously known as '''TCEB1-mutated renal cell carcinoma''' and '''renal cell carcinoma with TCEB1 mutation'''. It should '''not''' be confused with [[TFEB renal cell carcinoma]], also known as ''renal tumour with t(6;11) translocation''. | ||
This entity is not recognized by the WHO classification of renal neoplasia. It probably represents the previously described [[renal angiomyoadenomatous tumour]],<ref name=pmid25676555/><ref name=pmid19471960>{{Cite journal | last1 = Michal | first1 = M. | last2 = Hes | first2 = O. | last3 = Kuroda | first3 = N. | last4 = Kazakov | first4 = DV. | last5 = Hora | first5 = M. | title = Difference between RAT and clear cell papillary renal cell carcinoma/clear renal cell carcinoma. | journal = Virchows Arch | volume = 454 | issue = 6 | pages = 719 | month = Jun | year = 2009 | doi = 10.1007/s00428-009-0788-9 | PMID = 19471960 }}</ref> that was lumped with [[clear cell papillary renal cell carcinoma]] in the [[Vancouver classification|Vancouver modification of the WHO classification]]. | This entity is not recognized by the WHO classification of renal neoplasia. It probably represents the previously described [[renal angiomyoadenomatous tumour]],<ref name=pmid25676555/><ref name=pmid19471960>{{Cite journal | last1 = Michal | first1 = M. | last2 = Hes | first2 = O. | last3 = Kuroda | first3 = N. | last4 = Kazakov | first4 = DV. | last5 = Hora | first5 = M. | title = Difference between RAT and clear cell papillary renal cell carcinoma/clear renal cell carcinoma. | journal = Virchows Arch | volume = 454 | issue = 6 | pages = 719 | month = Jun | year = 2009 | doi = 10.1007/s00428-009-0788-9 | PMID = 19471960 }}</ref> that was lumped with [[clear cell papillary renal cell carcinoma]] in the [[Vancouver classification|Vancouver modification of the WHO classification]]. |
Revision as of 21:48, 11 October 2022
ELOC-mutated renal cell carcinoma | |
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Diagnosis in short | |
RCC with clear cells and fibromuscular band - morphology compatible with TCEB1 mutation. H&E stain. (WC) | |
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LM | clear cells with abundant cytoplasm and basal nuclei (may have luminal nuclei), usually ISUP nucleolar grade 2, fibromuscular bands |
LM DDx | clear cell renal cell carcinoma, clear cell tubulopapillary renal cell carcinoma |
IHC | CK7 +ve, CD10 +ve, CK34betaE12 -ve |
Molecular | TCEB1 mutation |
Site | kidney - see kidney tumours |
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Syndromes | possibly tuberous sclerosis - see tuberous sclerosis-associated renal cell carcinoma |
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Prevalence | very rare - evolving entity |
Prognosis | good - based on limited data |
ELOC-mutated renal cell carcinoma is a type of renal cell carcinoma that is morphologically similar to clear cell renal cell carcinoma and clear cell tubulopapillary renal cell carcinoma.[1] It was formally recognized in the WHO 2022 classification of renal neoplasia.[citation needed]
It was previously known as TCEB1-mutated renal cell carcinoma and renal cell carcinoma with TCEB1 mutation. It should not be confused with TFEB renal cell carcinoma, also known as renal tumour with t(6;11) translocation.
This entity is not recognized by the WHO classification of renal neoplasia. It probably represents the previously described renal angiomyoadenomatous tumour,[1][2] that was lumped with clear cell papillary renal cell carcinoma in the Vancouver modification of the WHO classification.
General
- Evolving entity.
- TCEB1 (transcription elongation factor B, polypeptide 1) is part of a complex that binds with the product of the VHL gene;[3] thus, it acts as a tumour suppressor.
- Good prognosis - based on limited data.[1]
Gross
- Cystic changes.
Microscopic
Features:
- Clear cells with abundant cytoplasm and basal nuclei.
- Luminal nuclei (like in clear cell tubulopapillary RCC) may be seen.
- Usually ISUP nucleolar grade 2.
- Fibromuscular bands - key feature.
DDx:
- Clear cell renal cell carcinoma.
- Clear cell tubulopapillary renal cell carcinoma - have luminal nuclei.
- Tuberous sclerosis-associated renal cell carcinoma.
Images
Morphology compatible
IHC
Features:[1]
- CK7 +ve.
- CD10 +ve.
- Typically negative in clear cell tubulopapillary renal cell carcinoma.
- CK34betaE12 -ve.
- Usually positive in clear cell tubulopapillary renal cell carcinoma.
Comparison between some renal tumours with clear cells
Tumour | CK7 | CD10 | CK34betaE12 (K903) |
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Clear cell renal cell carcinoma | -ve (may be focal) | +ve | -ve |
Clear cell tubulopapillary renal cell carcinoma | +ve (diffuse) | -ve | +ve |
TCEB1-mutated clear cell renal cell carcinoma | +ve | +ve | -ve |
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Molecular lacking
Right Kidney, Radical Nephrectomy: - CLEAR CELL RENAL CELL CARCINOMA with cysts and leiomyomatous stroma, see comment. -- Margin clear. -- Please see synoptic report for details. Comment: The tumour stains as follows: POSITIVE: CD10 (moderate), EMA (patchy), CAM5.2, CK7 (focal), PAX8 (diffuse). NEGATIVE: CK34betaE12, CK20. The staining and morphology raises the possibility of a TCEB1 mutation, described in a subset of clear cell renal cell carcinoma.[1] 1. Mod Pathol 28 (6): 845-53. doi:10.1038/modpathol.2015.6. PMID 25676555.
See also
- Vancouver classification.
- Renal angiomyoadenomatous tumour (renal cell carcinoma with angioleiomyoma-like stroma).
References
- ↑ 1.0 1.1 1.2 1.3 Hakimi, AA.; Tickoo, SK.; Jacobsen, A.; Sarungbam, J.; Sfakianos, JP.; Sato, Y.; Morikawa, T.; Kume, H. et al. (Jun 2015). "TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype.". Mod Pathol 28 (6): 845-53. doi:10.1038/modpathol.2015.6. PMID 25676555.
- ↑ Michal, M.; Hes, O.; Kuroda, N.; Kazakov, DV.; Hora, M. (Jun 2009). "Difference between RAT and clear cell papillary renal cell carcinoma/clear renal cell carcinoma.". Virchows Arch 454 (6): 719. doi:10.1007/s00428-009-0788-9. PMID 19471960.
- ↑ URL: http://www.omim.org/entry/600788. Accessed on: 12 June 2015.