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Updated: Dec 28, 2025
A Syngeneic Mouse Model of Metastatic Renal Cell Carcinoma for Quantitative and Longitudinal Assessment of Preclinical Therapies
Published on: April 12, 2017
[MiT family translocation renal cell carcinomas: Natural history, molecular features and multidisciplinary
Hugo Herrscher1, Alice Boilève2, Véronique Lindner3
1Hôpitaux universitaires de Strasbourg, service d'oncologie médicale, 67200 Strasbourg, France.
Abstract:
MiT family translocation renal cell carcinomas (tRCC) represent a rare subtype of renal cell carcinomas. These tumors have been introduced for the first time in the World Health Classification (WHO) classification of kidney cancers in 2004. tRCC are characterized by reccurent translocations involving members of the MiT family transcription factors, mainly TFE3 and TFEB. The estimated incidence of these tumors is ∼1-5 % among all renal cell carcinomas, with female prodominance. tRCC were initially described in children, and the spectrum has been expanded over time to encompass adolescents and adults. TFE3- and TFEB-rearranged RCC harbor characteristic clinicopathological and immunohistochemical features and fluorescent hybridization in situ is considered the gold standard for their diagnosis, although it has some limitations especially when the partners are located in the vicinity of TFE3. Nephron-sparing surgery is an efficient treatment of localized cases when achievable. In metastatic setting, targeted agents and immunotherapy showed modest efficacy, with response rates and median overall survival inferior to those observed in clear-cell renal cell carcinomas. Management of tRCC necessite a multidisciplinary team and accrual in clinical trials have to be encouraged when possible. Novel biological insights are urgently awaited to better understand the mechanisms associated with kidney oncogenesis in this setting, and ultimately help to identify therapeutic targets.
Insights
MiT family translocation renal cell carcinomas (tRCC) are rare kidney cancers characterized by specific gene rearrangements. Current treatments show limited efficacy, highlighting the need for new therapeutic targets.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- MiT family translocation renal cell carcinomas (tRCC) are a rare subtype of kidney cancer, first recognized in the 2004 WHO classification.
- These tumors are defined by translocations involving MiT family transcription factors, primarily TFE3 and TFEB.
- tRCC accounts for approximately 1-5% of all renal cell carcinomas and shows a female predominance.
Purpose of the Study:
- To provide an overview of MiT family translocation renal cell carcinomas.
- To discuss diagnostic methods, treatment strategies, and challenges in managing tRCC.
- To emphasize the need for further research into the underlying mechanisms and therapeutic targets.
Main Methods:
- Review of existing literature on MiT family translocation renal cell carcinomas.
- Discussion of diagnostic techniques, including fluorescent in situ hybridization (FISH).
- Analysis of current treatment outcomes for localized and metastatic tRCC.
Main Results:
- tRCC exhibit distinct clinicopathological and immunohistochemical features.
- Fluorescent in situ hybridization (FISH) is the gold standard for diagnosis, despite limitations.
- Nephron-sparing surgery is effective for localized disease; targeted agents and immunotherapy have shown modest efficacy in metastatic settings.
Conclusions:
- Management of tRCC requires a multidisciplinary approach.
- Clinical trial enrollment is crucial for advancing treatment options.
- Further research is essential to understand kidney oncogenesis in tRCC and identify novel therapeutic targets.
