MiT family translocation renal cell carcinomas: A 15th anniversary update.

MiT family translocation renal cell carcinomas: A 15th anniversary update. Histol Histopathol. 2019 Sep 06;:18159 Authors: Gandhi JS, Malik F, Amin MB, Argani P, Bahrami A Abstract Microphthalmia (MiT) family translocation renal cell carcinomas (RCCs) are a heterogeneous category of renal tumors which all express MiT transcription factors, typically from chromosomal translocation and rarely from gene amplification. This tumor family has two major subtypes [i.e., Xp11 translocation RCC and t(6;11) RCC] and several related neoplasms (i.e., TFEB amplification RCC and melanotic Xp11 translocation renal cancers). Increased understanding of the clinical, pathological, molecular and prognostic heterogeneity of these tumors, since their official recognition in 2004, provides the opportunity to identify prognostic biomarkers and to understand the reasons for tumor aggression. We will review the literature from the past 15 years and highlight the need for a greater understanding of the molecular mechanisms underpinning heterogeneous tumor behavior. PMID: 31489603 [PubMed - as supplied by publisher]
Source: Histology and Histopathology - Category: Cytology Tags: Histol Histopathol Source Type: research

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Conclusion: In this patient cohort, pazopanib was associated with inferior OS in comparison with sunitinib for metastatic nccRCC. Larger trials are ideally warranted to confirm these results. PMID: 31921344 [PubMed]
Source: Ecancermedicalscience - Category: Cancer & Oncology Tags: Ecancermedicalscience Source Type: research
To investigate the contrast-enhanced ultrasound (CEUS) findings of renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2/TFE3) in adult patients by comparison with those of c...
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Source: Genes and Development - Category: Genetics & Stem Cells Authors: Tags: Research Papers Source Type: research
Clauditz TS Abstract BACKGROUND AND METHODS: 8p deletions are common in renal cell carcinoma. To study their prognostic impact and association with kidney cancer phenotype, a tissue microarray with 1,809 cancers was analyzed by fluorescence in situ hybridization for 8p21 copy numbers. RESULTS: One thousand four hundred and seventy four interpretable tumors showed substantial differences between renal cancer subtypes. That 8p deletion was only seen in 1 (0.5%) of 216 papillary carcinomas underscores the biologic uniqueness of papillary kidney cancer, which is also defined by a highly distinct morphology. 8p de...
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CONCLUSION: Nivolumab demonstrated activity in unclassified nccRCC and ccRCC with>20% rhabdoid; further randomized clinical trials are warranted. IMPLICATIONS FOR PRACTICE: This article reports on the clinical activity and safety of immune checkpoint inhibitors in non-clear cell kidney cancer. The retrospective data with the meta-analysis provides a summary that will help guide the treatment of this rare and heterogeneous group of kidney cancers. PMID: 31501271 [PubMed - as supplied by publisher]
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CONCLUSIONS: These data revealed that TFEB mediates resistance to mTOR inhibition via induction of PD-L1 in human primary RCC tumors, RCC cells and murine xenograft model. Our data provide a strong rationale to target mTOR and PD-L1 jointly as a novel immunotherapeutic approach for RCC treatment. PMID: 31383732 [PubMed - as supplied by publisher]
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
ido Martignoni The new category of MiT family translocation renal cell carcinoma has been included into the World Health Organization (WHO) classification in 2016. The MiT family translocation renal cell carcinoma comprises Xp11 translocation renal cell carcinoma harboring TFE3 gene fusions and t(6;11) renal cell carcinoma harboring TFEB gene fusion. At the beginning, they were recognized in childhood; nevertheless, it has been demonstrated that these neoplasms can occur in adults as well. In the nineties, among Xp11 renal cell carcinoma, ASPL, PRCC, and SFPQ (PSF) were the first genes recognized as partners in TFE3 re...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
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Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Translocation renal cell carcinomas (tRCC) encompass a diverse group of tumors affecting younger patients, and often progressing as metastases. Accurate diagnosis is required to guide clinical management. Morphologic and immunohistochemical findings can prompt reflex FISH studies, but with several significant limitations. A better molecular assay is needed.
Source: Cancer Genetics and Cytogenetics - Category: Genetics & Stem Cells Authors: Source Type: research
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