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TFEB-rearranged renal cell carcinoma

MedGen UID:
1664261
•Concept ID:
C4745249
•
Neoplastic Process
Synonyms: Renal Cell Carcinoma with t(6;11);(p21;q12); MALAT1-TFEB; Renal Cell Carcinoma with t(6;11);(p21;q12); MALAT1::TFEB; t(6;11) Renal Cell Carcinoma; t(6;11);(p21;q12) Renal Cell Carcinoma; TFEB-Altered Renal Cell Carcinoma; TFEB-Rearranged Renal Cell Carcinoma
 
Monarch Initiative: MONDO:0958302

Definition

A rare renal cell carcinoma affecting young patients. It is characterized by the presence of the chromosomal translocation t(6;11) which fuses the TFEB transcription factor gene, located on chromosome 6, with the MALAT1 gene, located on chromosome 11. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTFEB-rearranged renal cell carcinoma

Professional guidelines

PubMed

The New WHO Category of "Molecularly Defined Renal Carcinomas": Clinical and Diagnostic Features and Management Implications.
Kanakaraj J, Chang J, Hampton LJ, Smith SC
Urol Oncol 2024 Jul;42(7):211-219. Epub 2024 Mar 21 doi: 10.1016/j.urolonc.2024.02.003. PMID: 38519377

Recent clinical studies

Diagnosis

TFE3 and TFEB-rearranged renal cell carcinomas: an immunohistochemical panel to differentiate from common renal cell neoplasms.
Caliò A, Marletta S, Brunelli M, Pedron S, Portillo SC, Segala D, Bariani E, Gobbo S, Netto G, Martignoni G
Virchows Arch 2022 Dec;481(6):877-891. Epub 2022 Aug 18 doi: 10.1007/s00428-022-03380-x. PMID: 35980471Free PMC Article

Prognosis

Sarcomatoid renal cell tumor harboring a novel BYSL::TFEB fusion with concurrent TFEB amplification.
Lin R, Flerova E, Wamsley CE, McCue PA, Lallas CD, Jiang W, Huang J, Wang ZX, Li L
Genes Chromosomes Cancer 2023 Jun;62(6):353-360. Epub 2023 Feb 3 doi: 10.1002/gcc.23125. PMID: 36704911
TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1.
Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G
Mod Pathol 2021 Apr;34(4):842-850. Epub 2020 Nov 18 doi: 10.1038/s41379-020-00713-6. PMID: 33208882

Clinical prediction guides

TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1.
Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G
Mod Pathol 2021 Apr;34(4):842-850. Epub 2020 Nov 18 doi: 10.1038/s41379-020-00713-6. PMID: 33208882

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