Difference between revisions of "ELOC-mutated renal cell carcinoma"

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ELOC-mutated renal cell carcinoma (view source)

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'''TCEB1-mutated renal cell carcinoma''', also '''renal cell carcinoma with TCEB1 mutation''', is an evolving entity that is 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>
{{ 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==
*Evolving entity.
*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]].
*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 and basal nuclei.  
*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'''.
*Fibromuscular bands - '''key feature'''.
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DDx:
DDx:
*[[Clear cell renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]].
*[[Clear cell tubulopapillary renal cell carcinoma]] - have luminal nuclei.
*[[Clear cell papillary renal cell tumour]] (previously ''clear cell papillary renal cell carcinoma'') - have luminal nuclei.
*[[Tuberous sclerosis-associated renal cell carcinoma]].
*[[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==
==IHC==
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*CK7 +ve.
*CK7 +ve.
*CD10 +ve.
*CD10 +ve.
**Typically negative in [[clear cell papillary renal cell tumour]].
*CK34betaE12 -ve.
*CK34betaE12 -ve.
**Usually positive in [[clear cell tubulopapillary renal cell carcinoma]].
**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==
Michael
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