Clinicopathologic Characteristics and Prognosis of Xp11.2 Translocation Renal Cell Carcinoma: Multicenter, Propensity Score Matching Analysis

Choo, Min Soo; Jeong, Chang Wook; Song, Cheryn; Jeon, Hwang Gyun; Seo, Seong Il; Hong, Sung Kyu; Byun, Seok‐Soo; Chung, Jae Eun; Hong, Sung‐Hoo; Hwang, Eu Chang; Kim, Hyeon Hoe; Kwak, Cheol

doi:10.1016/j.clgc.2017.04.015

Cited by 17 publications

(28 citation statements)

References 20 publications

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“…This single-center, retrospective study identi ed an overall incidence of 0.48% for XP11.2 translocation RCC out of all adult RCCs based on a ten-year data. This result was consistent with another Asian cohort [5] (0.72% in Korea) which further demonstrated the rarity of this disease in adults.…”

Section: Discussionsupporting

confidence: 92%

“…XP11.2 RCC accounts for 20 -40% of pediatric RCC [13] and only 0.72-1.6% of adult RCC [5,14] . This subtype of RCC in adults requires special attention and more intensive researches for its rarity, aggressiveness in nature [3,13] and possible different treatment options (eg.…”

Section: Discussionmentioning

confidence: 99%

“…Xp11.2 translocation renal cell carcinoma (XP11.2 RCC) is a rare and unique subtype of renal cell carcinoma characterized by translocations involving TFE3 gene [1][2][3] . It has been classi ed as a distinct clinical entity in the World Health Organization renal tumor classi cation (2004) and is now regarded as an important subtype of RCC, especially in children [4,5] . Compared to other subtypes of RCC, this malignancy manifests with more aggressive clinicopathologic features at diagnosis [5] and worse prognosis [3,6] .…”

Section: Introductionmentioning

confidence: 99%

“…It has been classi ed as a distinct clinical entity in the World Health Organization renal tumor classi cation (2004) and is now regarded as an important subtype of RCC, especially in children [4,5] . Compared to other subtypes of RCC, this malignancy manifests with more aggressive clinicopathologic features at diagnosis [5] and worse prognosis [3,6] . In regard to the risk of disease re-occurrence and death, it is important to carry out prognostic studies to identify potential preoperative risk factors for disease progression and death which might help to guide interventions in the future.…”

Section: Introductionmentioning

confidence: 99%

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Preoperative Plasma Fibrinogen Level: An Independent Predictor for Survival in Adult Patients With Xp11.2 Translocation Renal Cell Carcinoma

Dong

et al. 2020

Preprint

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BackgroundXp11.2 translocation renal cell carcinoma (Xp11.2 RCC) is a rare malignancy which is more common in children than in adults. It manifests with an aggressive course in adults and relatively indolent in children. Prognostic studies for adult patients are scare for the rarity of the disease; and the prognostic value of preoperative plasma fibrinogen level awaits further illumination.MethodsThis retrospective single-center study enrolled 24 consecutive newly diagnosed Xp11.2 RCC adult patients. Clinical presentations, baseline laboratory results and follow-up data were collected. Possible risk factors for progression free survival (PFS) and overall survival (OS) were first scanned with chi-square tests and t-tests to compare patients who suffered from progression or death and who did not. Independent risk factors for survival were further investigated with multivariate Cox regression.ResultsTwenty-four adult patients (median age 32, range 16-73), with a male-to-female ratio of 12:12, was included from 2010.4 to 2020.3. After a mean follow-up of 35.7months, seven patients died. With univariate analysis, higher C-reactive protein-to-albumin ratio (p=0.028), higher baseline fibrinogen level (p=0.006), and presence of distant metastasis(p=0.007) were associated with progression of disease; higher preoperative fibrinogen level (p=0.014) and distant metastasis (p= 0.020) were associated with death. With multivariate Cox regression, only baseline fibrinogen level (p=0.001) was identified as an independent risk factor for progression free survival; meanwhile, fibrinogen level (p= 0.048) and distant metastasis (p= 0.043) were identified as independent risk factors for survival.ConclusionsPreoperative plasma fibrinogen level, a routinely tested parameter before surgery, is a promising tool for risk stratification in adult patients with Xp11.2 RCC.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Preoperative Plasma Fibrinogen Level: An Independent Predictor for Survival in Adult Patients With Xp11.2 Translocation Renal Cell Carcinoma

Dong

et al. 2020

Preprint

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“…Xp11.2 translocation/TFE3 gene fusions renal cell carcinoma (Xp11.2 tRCC) was first listed as a new type of renal cell carcinoma (RCC) in the 2004 by the World Health Organization (WHO). Since then, it has received wide attention [ 1 – 5 ]. Xp11.2 tRCC is characterized by various translocations of the transcription factor E3(TFE3) on chromosome Xp11.2, resulting in overexpression of the TFE3 protein [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Preoperative neutrophil-to-lymphocyte ratio predicts the surgical outcome of Xp11.2 translocation/TFE3 renal cell carcinoma patients

Agizamhan

Gan

et al. 2018

BMC Urol

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BackgroundThe preoperative neutrophil-to-lymphocyte ratio (NLR), C-reactive protein/albumin ratio (CRP/Alb ratio) and platelet-to-lymphocyte ratio (PLR) have been demonstrated to predict the clinical outcome of various human cancer, including renal cell carcinoma(RCC). The aim of our study was to explore the prognostic values of these ratios in patients with Xp11.2 translocation/TFE3 gene fusions renal cell carcinoma (Xp11.2 tRCC).MethodsA retrospective multicentre study was performed in 82 Xp11.2 tRCC patients who underwent radical or partial nephrectomy. The optimal cutoff values of the NLR, CRP/Alb ratio and PLR were determined by the receiver operating characteristic (ROC) analysis. The impact of the NLR, CRP/Alb ratio and PLR, as well as other clinicopathological characteristics, on disease-free survival (DFS) and overall survival (OS) were evaluated using the univariate and multivariate Cox regression analyses.ResultsThe optimal cutoff values of the NLR, CRP/Alb ratio and PLR were set at 2.45, 140 and 0.08, respectively, according to the ROC analysis. Univariate analyses showed that the NLR, CRP/Alb ratio and PLR all were associated with DFS of Xp11.2 tRCC patients (P < 0.001, P = 0.005 and P = 0.001, respectively) and OS of Xp11.2 tRCC patients (P = 0.016, P = 0.003 and P = 0.014, respectively). Multivariate analysis indicated that the NLR was independently associated with DFS of Xp11.2 tRCC patients (hazard ratio [HR]: 4.25; 95% confidence interval [95% CI]: 1.19–15.18; P = 0.026) along with age (P = 0.004), the pT status (P < 0.001) and the pN status (P < 0.019), and the NLR (HR: 26.26; 95% CI: 1.44–480.3; P = 0.028) also was independently associated with OS in patients with Xp11.2 tRCC, along with age (P = 0.016) and a tumour thrombus (P = 0.007).ConclusionOverall, relatively high NLRs, CRP/Alb ratios and PLRs were associated with a poor prognosis of Xp11.2 tRCC patients; among of them, only the NLR independently predicted the progression of Xp11.2 tRCC, and the NLR may help to identify patients with high metastasis or relapse risk.

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Phenotypical screening on metastatic PRCC-TFE3 fusion translocation renal cell carcinoma organoids reveals potential therapeutic agents

Cao

Lan²,

Shang

et al. 2022

Clin Transl Oncol

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Purpose Translocation renal cell carcinoma (tRCC) is a subtype that occurs predominantly in children and young individuals. Metastatic tRCC occurring in young patients is more aggressive than that occurring in older patients, and there are still no effective therapies. Organoids can mimic original tissues and be assessed by high-throughput screening (HTS). We aimed to utilize patient-derived organoids and HTS to screen drugs that can be repurposed for metastatic tRCC with PRCC-TFE3 fusion. Methods Tumor tissues were obtained from treatment-naïve metastatic tRCC patients who underwent surgery. Histopathology and fluorescence in situ hybridization (FISH) confirmed the tRCC. Organoids derived from the dissected tissues were cultured and verified by FISH and RNA-seq. HTS was performed to seek promising drugs, and potential mechanisms were explored by RNA-seq and cell-based studies. Results We successfully established a metastatic tRCC organoid with PRCC-TFE3 fusion, a common fusion subtype, and its characteristics were verified by histopathology, FISH, and RNA-seq. An HTS assay was developed, and the robustness was confirmed. A compound library of 1816 drugs was screened. Eventually, axitinib, crizotinib, and JQ-1 were selected for further validation and were found to induce cell cycle arrest and apoptosis. RNA-seq analyses of posttreatment organoids indicated that crizotinib induced significant changes in autophagy-related genes, consistent with the potential pathogenesis of tRCC. Conclusions We established and validated organoids derived from tissues dissected from a patient with metastatic tRCC with PRCC-TFE3 fusion and achieved the HTS process for the first time. Crizotinib might be a targeted therapy worthy of exploration in the clinic for metastatic tRCC with PRCC-TFE3 fusion. Such organoid and HTS assays may represent a promising model system in translational research assisting in the development of clinical strategies.

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Clinicopathologic Characteristics and Prognosis of Xp11.2 Translocation Renal Cell Carcinoma: Multicenter, Propensity Score Matching Analysis

Cited by 17 publications

References 20 publications

Preoperative Plasma Fibrinogen Level: An Independent Predictor for Survival in Adult Patients With Xp11.2 Translocation Renal Cell Carcinoma

Preoperative Plasma Fibrinogen Level: An Independent Predictor for Survival in Adult Patients With Xp11.2 Translocation Renal Cell Carcinoma

Preoperative neutrophil-to-lymphocyte ratio predicts the surgical outcome of Xp11.2 translocation/TFE3 renal cell carcinoma patients

Phenotypical screening on metastatic PRCC-TFE3 fusion translocation renal cell carcinoma organoids reveals potential therapeutic agents

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