Xp11.2 translocation renal neoplasm with features of TFE3 rearrangement associated renal cell carcinoma and Xp11 translocation renal mesenchymal tumor with melanocytic differentiation harboring NONO-TFE3 fusion gene.

Xp11.2 translocation renal neoplasm with features of TFE3 rearrangement associated renal cell carcinoma and Xp11 translocation renal mesenchymal tumor with melanocytic differentiation harboring NONO-TFE3 fusion gene. Pathol Res Pract. 2019 Jun 27;:152521 Authors: Yu W, Wang Y, Rao Q, Jiang Y, Zhang W, Li Y Abstract Xp11.2 translocation/TFE3 rearrangement-associated renal cell carcinoma (RCC) and Xp11 translocation renal mesenchymal tumor are distinct tumor entity. To broaden the spectrum of Xp11 neoplasms, we investigated a novel tumor exhibiting morphologies overlapping Xp11.2 translocation/TFE3 rearrangement-associated RCC and the mesenchymal counterpart with melanocytic differentiation by immunohistochemistry, fluorescence in situ hybridization (FISH) and RNA sequencing, as well as literature review. Histologically, the tumor was composed of three different types of tumor cells, including a large proportion of clear cells, small round cells, and a few spindle cells, presenting a relatively clear border in the majority area. The nuclei of all tumor cells showed extensively and strong positive expressions of TFE3. Whereas, the clear cells positively expressed the RCC-related markers including PAX8, RCC marker and CD10, and negatively expressed HMB45; On the contrary, the small round cells and spindle cells positively expressed melanocytic marker HMB45, and negatively expressed PAX8, RCC marker and CD10. The ki67 index was higher in the small round cells...
Source: Pathology, Research and Practice - Category: Pathology Authors: Tags: Pathol Res Pract Source Type: research

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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]
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Oncologist Source Type: research
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 the...
Source: Histology and Histopathology - Category: Cytology Tags: Histol Histopathol Source Type: research
Publication date: 3 September 2019Source: Cell Reports, Volume 28, Issue 10Author(s): Wen-Hsuan Yang, Chien-Kuang Cornelia Ding, Tianai Sun, Gabrielle Rupprecht, Chao-Chieh Lin, David Hsu, Jen-Tsan ChiSummaryDespite recent advances, the poor outcomes in renal cell carcinoma (RCC) suggest novel therapeutics are needed. Ferroptosis is a form of regulated cell death, which may have therapeutic potential toward RCC; however, much remains unknown about the determinants of ferroptosis susceptibility. We found that ferroptosis susceptibility is highly influenced by cell density and confluency. Because cell density regulates the H...
Source: Cell Reports - Category: Cytology Source Type: research
The aim of the study was to report the experience and outcomes of Xp11.2 translocation renal cell carcinoma (tRCC) patients with tumor thrombus undergoing radical nephrectomy and thrombectomy. Between January 2017 and December 2017, 66 consecutive patients with RCC and venous thrombus involvement received surgical treatment at Peking University Third Hospital. Of which, 5 patients were confirmed of Xp11.2 tRCC, 61 patients were diagnosed of non-tRCC subtypes including 45 ccRCCs, 10 pRCCs, and 6 other subtypes. Demographic, clinical, operation, pathological and follow-up data were extracted for analysis. Prognostic factors...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
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
Purpose of review To review the presentation, natural history and treatment of renal cell carcinoma in children and young adults with renal cell carcinoma (RCC). Recent findings Complete resection of lymph nodes at the time of tumor resection can improve clinical outcomes and limit the need for adjuvant chemotherapy. Genetic alterations that lead to translocation tumors are a therapeutic target of receptor tyrosine kinase inhibitors. Summary The incidence of RCC increases with age. Unlike adult patients, young patients with RCC present symptomatically and at higher stage and grade. Translocation tumors predominate ...
Source: Current Opinion in Urology - Category: Urology & Nephrology Tags: PAEDIATRIC URO-ONCOLOGY: Edited by Manuela P. Hiess Source Type: research
Publication date: Available online 27 June 2019Source: Pathology - Research and PracticeAuthor(s): Wenjuan Yu, Yuewei Wang, Qiu Rao, Yanxia Jiang, Wei Zhang, Yujun LiAbstractXp11.2 translocation/TFE3 rearrangement-associated renal cell carcinoma (RCC) and Xp11 translocation renal mesenchymal tumor are distinct tumor entity. To broaden the spectrum of Xp11 neoplasms, we investigated a novel tumor presenting morphology overlapping Xp11.2 translocation/TFE3 rearrangement-associated RCC and the mesenchymal counterpart with melanocytic differentiation by immunohistochemistry, Fluorescence in situ hybridization (FISH) and RNA se...
Source: Pathology Research and Practice - Category: Pathology Source Type: research
Modern Pathology, Published online: 07 June 2019; doi:10.1038/s41379-019-0283-zXp11 translocation renal cell carcinoma and clear cell renal cell carcinoma with TFE3 strong positive immunostaining: morphology, immunohistochemistry, and FISH analysis
Source: Modern Pathology - Category: Pathology Authors: Source Type: research
Publication date: Available online 26 May 2019Source: Pathology - Research and PracticeAuthor(s): Noriyoshi Ishikawa, Mamiko Nagase, Saki Takami, Nahoko Nagano, Asuka Araki, Teruaki Iwahashi, Naotaka Yamauchi, Taku Yamasaki, Hiroaki Shiina, Riruke MaruyamaAbstractXp11.2 translocation renal cell carcinoma (Xp11tRCC) is a subtype of renal cell carcinoma (RCC) characterized by chromosomal rearrangement of the region harboring the transcription factor for immunoglobulin heavy-chain enhancer 3 (TFE3). Xp11tRCCs comprises 20% to 40% of RCCs of children and adolescents and is generally associated with good prognosis. However in a...
Source: Pathology Research and Practice - Category: Pathology Source Type: research
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