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MIT family translocation renal cell carcinoma

MedGen UID:
1376834
•Concept ID:
C4518356
•
Neoplastic Process
Synonyms: Carcinoma associated with MITF/TFE translocation; carcinoma associated with MITF/TFE translocation; MiT family translocation renal cell carcinoma; MiT Family Translocation-Associated Renal Cell Carcinoma; Renal Cell Carcinoma with MiT Translocations; Translocation renal cell carcinoma; translocation renal cell carcinoma
SNOMED CT: MiT family translocation renal cell carcinoma (733881007); Translocation renal cell carcinoma (764694005); MiT family translocation renal cell carcinoma (764694005)
 
Monarch Initiative: MONDO:0017886
Orphanet: ORPHA319308

Definition

MiT family translocation renal cell carcinoma (t-RCC) is a rare subtype of renal cell carcinoma with recurrent genetic abnormalities, harboring rearrangements of the <i>TFE3</i> (Xp11 t-RCC) or <i>TFEB</i> [t(6;11) t-RCC] genes. The t(6;11) t-RCC has distinctive histologic features of biphasic appearance with larger epitheloid and smaller eosinophilic cells. The symptoms are usually non-specific and include hematuria, flank pain, palpable abdominal mass and/or systemic symptoms of anemia, fatigue and fever. [from ORDO]

Professional guidelines

PubMed

Systemic Therapies for the Management of Non-Clear Cell Renal Cell Carcinoma: What Works, What Doesn't, and What the Future Holds.
Zoumpourlis P, Genovese G, Tannir NM, Msaouel P
Clin Genitourin Cancer 2021 Apr;19(2):103-116. Epub 2020 Dec 2 doi: 10.1016/j.clgc.2020.11.005. PMID: 33358151Free PMC Article

Recent clinical studies

Etiology

MiT/TFE Family Renal Cell Carcinoma.
Tang J, Baba M
Genes (Basel) 2023 Jan 5;14(1) doi: 10.3390/genes14010151. PMID: 36672892Free PMC Article
Immune Checkpoint Therapy Combinations in Adult Advanced MiT Family Translocation Renal Cell Carcinomas.
Alhalabi O, Thouvenin J, Négrier S, Vano YA, Campedel L, Hasanov E, Bakouny Z, Hahn AW, Bilen MA, Msaouel P, Choueiri TK, Viswanathan SR, Sircar K, Albiges L, Malouf GG, Tannir NM
Oncologist 2023 May 8;28(5):433-439. doi: 10.1093/oncolo/oyac262. PMID: 36640141Free PMC Article
Efficacy of Cabozantinib in Metastatic MiT Family Translocation Renal Cell Carcinomas.
Thouvenin J, Alhalabi O, Carlo M, Carril-Ajuria L, Hirsch L, Martinez-Chanza N, Négrier S, Campedel L, Martini D, Borchiellini D, Chahoud J, Lodi M, Barthélémy P, Hasanov E, Hahn AW, Gil T, Viswanathan SR, Bakouny Z, Msaouel P, Asim Bilen M, Choueiri TK, Albiges L, Tannir NM, Malouf GG
Oncologist 2022 Dec 9;27(12):1041-1047. doi: 10.1093/oncolo/oyac158. PMID: 35979929Free PMC Article
Advanced nccRCC: what therapeutic options in 2022?
Bigot C, Boudier P, Ladoire S, Barthélémy P
Bull Cancer 2022 May;109(2S):2S39-2S46. doi: 10.1016/S0007-4551(22)00237-5. PMID: 35760469
MiT family translocation renal cell carcinoma.
Argani P
Semin Diagn Pathol 2015 Mar;32(2):103-13. Epub 2015 Feb 4 doi: 10.1053/j.semdp.2015.02.003. PMID: 25758327

Diagnosis

MiT/TFE Family Renal Cell Carcinoma.
Tang J, Baba M
Genes (Basel) 2023 Jan 5;14(1) doi: 10.3390/genes14010151. PMID: 36672892Free PMC Article
Proteogenomic characterization of MiT family translocation renal cell carcinoma.
Qu Y, Wu X, Anwaier A, Feng J, Xu W, Pei X, Zhu Y, Liu Y, Bai L, Yang G, Tian X, Su J, Shi GH, Cao DL, Xu F, Wang Y, Gan HL, Ni S, Sun MH, Zhao JY, Zhang H, Ye D, Ding C
Nat Commun 2022 Dec 5;13(1):7494. doi: 10.1038/s41467-022-34460-w. PMID: 36470859Free PMC Article
Efficacy of Cabozantinib in Metastatic MiT Family Translocation Renal Cell Carcinomas.
Thouvenin J, Alhalabi O, Carlo M, Carril-Ajuria L, Hirsch L, Martinez-Chanza N, Négrier S, Campedel L, Martini D, Borchiellini D, Chahoud J, Lodi M, Barthélémy P, Hasanov E, Hahn AW, Gil T, Viswanathan SR, Bakouny Z, Msaouel P, Asim Bilen M, Choueiri TK, Albiges L, Tannir NM, Malouf GG
Oncologist 2022 Dec 9;27(12):1041-1047. doi: 10.1093/oncolo/oyac158. PMID: 35979929Free PMC Article
Systemic Therapies for the Management of Non-Clear Cell Renal Cell Carcinoma: What Works, What Doesn't, and What the Future Holds.
Zoumpourlis P, Genovese G, Tannir NM, Msaouel P
Clin Genitourin Cancer 2021 Apr;19(2):103-116. Epub 2020 Dec 2 doi: 10.1016/j.clgc.2020.11.005. PMID: 33358151Free PMC Article
New entities, new technologies, new findings: A review of the cytologic features of recently established subtypes of renal cell carcinoma.
Robila V, Kraft AO, Smith SC
Cancer Cytopathol 2019 Feb;127(2):79-97. Epub 2019 Jan 28 doi: 10.1002/cncy.22093. PMID: 30690877

Therapy

MiT family translocation renal cell carcinoma in an elderly male.
Prachi, Aiyer HM
Indian J Pathol Microbiol 2021 Jul-Sep;64(3):553-555. doi: 10.4103/IJPM.IJPM_365_20. PMID: 34341271
Secondary renal neoplasia following chemotherapy or radiation in pediatric patients.
Gupta S, Vanderbilt CM, Leibovich BC, Herrera-Hernandez L, Raghunathan A, Sukov WR, Voss JS, Barr Fritcher EG, Reed KA, Lohse CM, Reuter VE, Jimenez RE, Thompson RH, Cheville JC
Hum Pathol 2020 Sep;103:1-13. Epub 2020 Jul 15 doi: 10.1016/j.humpath.2020.07.014. PMID: 32681944Free PMC Article

Prognosis

Proteogenomic characterization of MiT family translocation renal cell carcinoma.
Qu Y, Wu X, Anwaier A, Feng J, Xu W, Pei X, Zhu Y, Liu Y, Bai L, Yang G, Tian X, Su J, Shi GH, Cao DL, Xu F, Wang Y, Gan HL, Ni S, Sun MH, Zhao JY, Zhang H, Ye D, Ding C
Nat Commun 2022 Dec 5;13(1):7494. doi: 10.1038/s41467-022-34460-w. PMID: 36470859Free PMC Article
Advanced nccRCC: what therapeutic options in 2022?
Bigot C, Boudier P, Ladoire S, Barthélémy P
Bull Cancer 2022 May;109(2S):2S39-2S46. doi: 10.1016/S0007-4551(22)00237-5. PMID: 35760469
Diagnostic utility of one-stop fusion gene panel to detect TFE3/TFEB gene rearrangement and amplification in renal cell carcinomas.
Harada S, Caliò A, Janowski KM, Morlote D, Rodriguez Pena MD, Canete-Portillo S, Harbi D, DeFrank G, Magi-Galluzzi C, Netto GJ, Martignoni G, Mackinnon AC
Mod Pathol 2021 Nov;34(11):2055-2063. Epub 2021 Jun 19 doi: 10.1038/s41379-021-00858-y. PMID: 34148064

Clinical prediction guides

Efficacy of Cabozantinib in Metastatic MiT Family Translocation Renal Cell Carcinomas.
Thouvenin J, Alhalabi O, Carlo M, Carril-Ajuria L, Hirsch L, Martinez-Chanza N, Négrier S, Campedel L, Martini D, Borchiellini D, Chahoud J, Lodi M, Barthélémy P, Hasanov E, Hahn AW, Gil T, Viswanathan SR, Bakouny Z, Msaouel P, Asim Bilen M, Choueiri TK, Albiges L, Tannir NM, Malouf GG
Oncologist 2022 Dec 9;27(12):1041-1047. doi: 10.1093/oncolo/oyac158. PMID: 35979929Free PMC Article
Diagnostic utility of one-stop fusion gene panel to detect TFE3/TFEB gene rearrangement and amplification in renal cell carcinomas.
Harada S, Caliò A, Janowski KM, Morlote D, Rodriguez Pena MD, Canete-Portillo S, Harbi D, DeFrank G, Magi-Galluzzi C, Netto GJ, Martignoni G, Mackinnon AC
Mod Pathol 2021 Nov;34(11):2055-2063. Epub 2021 Jun 19 doi: 10.1038/s41379-021-00858-y. PMID: 34148064
Stimulator of interferon genes (STING) immunohistochemical expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney.
Caliò A, Brunelli M, Gobbo S, Pedron S, Segala D, Argani P, Martignoni G
Pathology 2021 Aug;53(5):579-585. Epub 2021 Jan 16 doi: 10.1016/j.pathol.2020.09.025. PMID: 33461798

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