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

From Libre Pathology
Jump to navigation Jump to search
Line 1: Line 1:
{{ 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. [[H&E stain]]. (WC)
| Synonyms  =
| 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 tubulopapillary renal cell carcinoma]]
| 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        =
}}
'''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>
'''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>


Line 6: Line 37:
*Evolving entity.
*Evolving entity.
*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==

Revision as of 21:49, 12 June 2015

ELOC-mutated renal cell carcinoma
Diagnosis in short

RCC with clear cells and fibromuscular band. H&E stain. (WC)
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
Syndromes possibly tuberous sclerosis - see tuberous sclerosis-associated renal cell carcinoma
Prevalence very rare - evolving entity
Prognosis good - based on limited data

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.[1]

It should not be confused with TFEB renal cell carcinoma, also known as renal tumour with t(6;11) translocation.

General

  • Evolving entity.
  • TCEB1 (transcription elongation factor B, polypeptide 1) is part of a complex that binds with the product of the VHL gene;[2] 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:

IHC

Features:[1]

See also

References

  1. ↑ 1.0 1.1 1.2 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.
  2. ↑ URL: http://www.omim.org/entry/600788. Accessed on: 12 June 2015.
Retrieved from "https://librepathology.org/w/index.php?title=ELOC-mutated_renal_cell_carcinoma&oldid=38514"