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''' | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Renal cell carcinoma with clear cells and fibromuscular bands -- intermed mag.jpg | |||
| Width = | |||
| Caption = RCC with clear cells and fibromuscular band - morphology compatible with TCEB1 mutation. [[H&E stain]]. (WC) | |||
| Synonyms = TCEB1-mutated renal cell carcinoma (old name) | |||
| Micro = clear cells with abundant cytoplasm and basal nuclei (may have luminal nuclei), usually [[ISUP nucleolar grade]] 2, fibromuscular bands | |||
| Subtypes = | |||
| LMDDx = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell tumour]] (clear cell papillary RCC), [[renal cell carcinoma with fibromyomatous stroma]] | |||
| Stains = | |||
| IHC = CK7 +ve, CD10 +ve, [[CK34betaE12]] -ve | |||
| EM = | |||
| Molecular = TCEB1 mutation | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[kidney]] - see ''[[kidney tumours]]'' | |||
| Assdx = | |||
| Syndromes = possibly [[tuberous sclerosis]] - see ''[[tuberous sclerosis-associated renal cell carcinoma]]'' | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = very rare - evolving entity | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = good - based on limited data | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''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 papillary renal cell tumour]] (previously ''clear cell papillary 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''. | ||
It is unrelated to 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]]. | |||
==General== | ==General== | ||
* | *Entity part of WHO Blue Book (5th Edition). | ||
*TCEB1 (transcription elongation factor B, polypeptide 1) is part of a complex that binds with the product of the [[VHL]] gene;<ref>URL: [http://www.omim.org/entry/600788 http://www.omim.org/entry/600788]. Accessed on: 12 June 2015.</ref> thus, it acts as a [[tumour suppressor]]. | |||
*Good prognosis - based on limited data.<ref name=pmid25676555/> | |||
==Gross== | ==Gross== | ||
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==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*Clear cells with abundant cytoplasm. | *Clear cells with abundant cytoplasm and basal nuclei. | ||
**Luminal nuclei (like in ''clear cell tubulopapillary RCC'') may be seen. | |||
*Usually [[ISUP nucleolar grade]] 2. | *Usually [[ISUP nucleolar grade]] 2. | ||
*Fibromuscular bands - '''key feature'''. | |||
DDx: | DDx: | ||
*[[Clear cell renal cell carcinoma]]. | *[[Clear cell renal cell carcinoma]]. | ||
*[[Clear cell | *[[Clear cell papillary renal cell tumour]] (previously ''clear cell papillary renal cell carcinoma'') - have luminal nuclei. | ||
*[[Tuberous sclerosis-associated renal cell carcinoma]]. | |||
*[[Renal cell carcinoma with fibromyomatous stroma]] has TSC/mTOR pathway mutation. | |||
===Images=== | |||
====Morphology compatible==== | |||
<gallery> | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- very low mag.jpg | RCC CCFB - very low mag. | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- low mag.jpg | RCC CCFB - low mag. | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- intermed mag.jpg | RCC CCFB - intermed. mag. | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- high mag.jpg | RCC CCFB - high mag. | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands - alt -- high mag.jpg | RCC CCFB - high mag. | |||
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- very high mag.jpg | RCC CCFB - very high mag. | |||
</gallery> | |||
==IHC== | |||
Features:<ref name=pmid25676555/> | |||
*CK7 +ve. | |||
*CD10 +ve. | |||
**Typically negative in [[clear cell papillary renal cell tumour]]. | |||
*CK34betaE12 -ve. | |||
**Usually positive in [[clear cell papillary renal cell tumour]]. | |||
Others: | |||
*[[GPNMB]] -ve. | |||
**TSC1/TSC2/MTOR mutated RCC is GPNMB +ve.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref> | |||
===Comparison between some renal tumours with clear cells=== | |||
{| class="wikitable sortable" | |||
!Tumour | |||
!CK7 | |||
!CD10 | |||
!CK34betaE12 (K903) | |||
![[GPNMB]] | |||
|- | |||
|Clear cell renal cell carcinoma | |||
| -ve (may be focal) | |||
| +ve | |||
| -ve | |||
| usu. -ve | |||
|- | |||
|Clear cell papillary renal cell tumour | |||
| +ve (diffuse) | |||
| -ve | |||
| +ve | |||
| -ve<ref name=pmid37661807/> | |||
|- | |||
|ELOC-mutated renal cell carcinoma | |||
| +ve | |||
| +ve | |||
| -ve | |||
| -ve | |||
|- | |||
|[[Renal cell carcinoma with fibromyomatous stroma|Renal cell carcinoma with fibromyomatous stroma and TSC/mTOR-mutation]] | |||
| +ve | |||
| +ve | |||
| -ve | |||
| +ve | |||
|- | |||
|} | |||
==Sign out== | |||
===Molecular lacking=== | |||
<pre> | |||
Right Kidney, Radical Nephrectomy: | |||
- RENAL CELL CARCINOMA with clear cells, 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), CK7 (diffuse), PAX8 (diffuse). | |||
NEGATIVE: CK34betaE12, CK20. | |||
The differential diagnosis includes: (1) clear cell renal cell carcinoma, (2) RCC with a mTOR pathway mutation, and (3) ELOC-mutated RCC. | |||
</pre> | |||
==See also== | ==See also== | ||
*[[Vancouver classification]]. | *[[Vancouver classification]]. | ||
*[[Renal angiomyoadenomatous tumour]] (renal cell carcinoma with angioleiomyoma-like stroma). | |||
*[[Renal cell carcinoma with fibromyomatous stroma]]. | |||
==References== | ==References== |
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