Z Zhang, M Lu, Y Sun, J Dong, X Hou, C Xiao, G Wang, X Tian, L Ma, H Zhang, S Zhang
{"title":"[tfe3重排肾细胞癌合并静脉肿瘤血栓的临床病理特征及生存分析]。","authors":"Z Zhang, M Lu, Y Sun, J Dong, X Hou, C Xiao, G Wang, X Tian, L Ma, H Zhang, S Zhang","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>To review the clinicopathological features of <i>TFE3</i>-rearranged renal cell carcinoma (<i>TFE3</i>-RCC) with venous tumor thrombus (VT) (<i>TFE3</i>-VT), to explore treatment strategies and to prognostic characteristics, and to provide diagnostic and therapeutic references for <i>TFE3</i>-VT patients.</p><p><strong>Methods: </strong>Patients who underwent surgery at Department of Urology, Peking University Third Hospital from January 2013 to January 2024 were enrolled, including three cohorts: Pathologically confirmed <i>TFE3</i>-VT patients, <i>TFE3</i>-RCC patients without VT (<i>TFE3</i>-non-VT), and non-<i>TFE3</i>-rearranged renal cell carcinoma patients with VT (non-<i>TFE3</i>-VT). Clinical history, imaging data, pathological data, and follow-up records were collected. Primary and secondary endpoints were progression-free survival (PFS) and overall survival (OS), respectively. (1) Baseline characteristics were compared between the <i>TFE3</i>-VT and <i>TFE3</i>-non-VT patients. Normally distributed continuous variables were expressed as mean±SD and compared using Student's <i>t</i>-test; non-normally distributed variables were expressed as <i>M</i> (<i>P</i><sub>25</sub>, <i>P</i><sub>75</sub>) and analyzed with Mann-Whitney <i>U</i> test; categorical variables were described as frequency and percentage [<i>n</i> (%)] and compared by <i>χ</i><sup>2</sup> test or Fisher's exact test. (2) Clinical history, radiological presentations, surgical data, and histopathological features of the <i>TFE3</i>-VT patients were comprehensively characterized. (3) Survival analysis was performed for the <i>TFE3</i>-VT patients. Follow-up data of the <i>TFE3</i>-VT patients were described in detail, and their survival outcomes were compared with the <i>TFE3</i>-non-VT and non-<i>TFE3</i>-VT patients. When compared with the <i>TFE3</i>-non-VT counterparts, Kaplan-Meier method was used to generate PFS and OS curves among: (1) the <i>TFE3</i>-RCC patients across clinical stages Ⅰ-Ⅳ; (2) <i>TFE3</i>-VT <i>versus</i> <i>TFE3</i>-non-VT cohorts; (3) stage Ⅲ subgroups of the <i>TFE3</i>-VT and <i>TFE3</i>-non-VT patients. Intergroup survival differences were statistically evaluated using Log-rank tests. For comparisons with the non-<i>TFE3</i>-VT patients, a 1 : 1 propensity score matching (PSM) was implemented to balance baseline characteristics between the two cohorts. Post-matching Kaplan-Meier curves were generated to compare PFS and OS between the matched groups, with Log-rank tests employed to determine statistical significance of survival disparities. All statistical analyses were conducted with R software (v 4.2.3), and two-tailed <i>P</i> < 0.05 was considered statistically significant.</p><p><strong>Results: </strong>The study included 45 <i>TFE3</i>-RCC patients: 13 <i>TFE3</i>-VT and 32 <i>TFE3</i>-non-VT cases. Additionally, 523 non-<i>TFE3</i>-VT patients were enrolled. Among the 13 <i>TFE3</i>-VT patients, 9 were female (69.2%) and 4 male (30.8%), with a mean age of (37.9±14.4) years, mean BMI of (22.2 ± 3.5) kg/m<sup>2</sup>, median age-adjusted Charlson comorbidity index (aCCI) of 1.0 (0.0, 1.0), and preoperative creatinine level of (75.3±15.9) μmol/L; tumors were located in the left kidney in 7 patients (53.8%) and right kidney in 6 (46.2%); preoperative distant metastasis (M1 stage) was present in 6 patients (46.2%), while 7 (53.8%) showed no metastasis; VT distribution by Mayo level comprised 7 cases (53.8%) at level 0, 1 case each at levels Ⅰ and Ⅳ (7.7% respectively), and 2 cases each at levels Ⅱ and Ⅲ (15.4% respectively); surgical approaches comprised open surgery (<i>n</i>=2, 15.4%), laparoscopic surgery (<i>n</i>=6, 46.1%), and robot-assisted laparoscopic surgery (<i>n</i>=5, 38.5%); mean operative time was (273±79) min, and intraoperative blood loss was (722±570) mL; mean maximum tumor diameter was (10.8±2.4) cm. All the 13 patients underwent TFE3 protein immunohistochemistry (IHC) staining, with 7 confirmed by fluorescence <i>in situ</i> hybridization (FISH). Tumor recurrence or metastasis occurred in 11 patients (84.6%), and 9 (69.2%) patients died during follow-up. Median PFS was 4 months (1 year PFS rate: 31%), and median OS was 13 months (1 year OS rate: 54%). Survival analysis of 45 <i>TFE3</i>-RCC patients revealed statistically significant differences in PFS and OS across all the clinical stages (<i>P</i> < 0.001). The <i>TFE3</i>-VT patients exhibited significantly worse PFS and OS than the <i>TFE3</i>-non-VT patients (<i>P</i> < 0.001), with persistent significance in stage Ⅲ subgroup analysis (<i>P</i> < 0.05). After PSM, <i>TFE3</i>-VT patients showed significantly inferior PFS compared with non-<i>TFE3</i>-VT (<i>P</i>=0.01), though no significant difference was shown between the OS curves (<i>P</i>=0.11).</p><p><strong>Conclusion: </strong><i>TFE3</i>-VT predominantly occurs in young females with frequent preoperative metastases. Strongly-positive staining of TFE3 protein in IHC staining and red-green split signals in FISH tests are reliable diagnostic markers. <i>TFE3</i>-VT patients exhibit inferior survival compared with <i>TFE3</i>-non-VT patients and earlier progression than non-<i>TFE3</i>-VT patients.</p>","PeriodicalId":8790,"journal":{"name":"北京大学学报（医学版）","volume":"57 4","pages":"650-661"},"PeriodicalIF":0.0000,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12340522/pdf/","citationCount":"0","resultStr":"{\"title\":\"[Clinicopathological features and survival analysis of <i>TFE3</i>-rearranged renal cell carcinoma with venous tumor thrombus].\",\"authors\":\"Z Zhang, M Lu, Y Sun, J Dong, X Hou, C Xiao, G Wang, X Tian, L Ma, H Zhang, S Zhang\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>To review the clinicopathological features of <i>TFE3</i>-rearranged renal cell carcinoma (<i>TFE3</i>-RCC) with venous tumor thrombus (VT) (<i>TFE3</i>-VT), to explore treatment strategies and to prognostic characteristics, and to provide diagnostic and therapeutic references for <i>TFE3</i>-VT patients.</p><p><strong>Methods: </strong>Patients who underwent surgery at Department of Urology, Peking University Third Hospital from January 2013 to January 2024 were enrolled, including three cohorts: Pathologically confirmed <i>TFE3</i>-VT patients, <i>TFE3</i>-RCC patients without VT (<i>TFE3</i>-non-VT), and non-<i>TFE3</i>-rearranged renal cell carcinoma patients with VT (non-<i>TFE3</i>-VT). Clinical history, imaging data, pathological data, and follow-up records were collected. Primary and secondary endpoints were progression-free survival (PFS) and overall survival (OS), respectively. (1) Baseline characteristics were compared between the <i>TFE3</i>-VT and <i>TFE3</i>-non-VT patients. Normally distributed continuous variables were expressed as mean±SD and compared using Student's <i>t</i>-test; non-normally distributed variables were expressed as <i>M</i> (<i>P</i><sub>25</sub>, <i>P</i><sub>75</sub>) and analyzed with Mann-Whitney <i>U</i> test; categorical variables were described as frequency and percentage [<i>n</i> (%)] and compared by <i>χ</i><sup>2</sup> test or Fisher's exact test. (2) Clinical history, radiological presentations, surgical data, and histopathological features of the <i>TFE3</i>-VT patients were comprehensively characterized. (3) Survival analysis was performed for the <i>TFE3</i>-VT patients. Follow-up data of the <i>TFE3</i>-VT patients were described in detail, and their survival outcomes were compared with the <i>TFE3</i>-non-VT and non-<i>TFE3</i>-VT patients. When compared with the <i>TFE3</i>-non-VT counterparts, Kaplan-Meier method was used to generate PFS and OS curves among: (1) the <i>TFE3</i>-RCC patients across clinical stages Ⅰ-Ⅳ; (2) <i>TFE3</i>-VT <i>versus</i> <i>TFE3</i>-non-VT cohorts; (3) stage Ⅲ subgroups of the <i>TFE3</i>-VT and <i>TFE3</i>-non-VT patients. Intergroup survival differences were statistically evaluated using Log-rank tests. For comparisons with the non-<i>TFE3</i>-VT patients, a 1 : 1 propensity score matching (PSM) was implemented to balance baseline characteristics between the two cohorts. Post-matching Kaplan-Meier curves were generated to compare PFS and OS between the matched groups, with Log-rank tests employed to determine statistical significance of survival disparities. All statistical analyses were conducted with R software (v 4.2.3), and two-tailed <i>P</i> < 0.05 was considered statistically significant.</p><p><strong>Results: </strong>The study included 45 <i>TFE3</i>-RCC patients: 13 <i>TFE3</i>-VT and 32 <i>TFE3</i>-non-VT cases. Additionally, 523 non-<i>TFE3</i>-VT patients were enrolled. Among the 13 <i>TFE3</i>-VT patients, 9 were female (69.2%) and 4 male (30.8%), with a mean age of (37.9±14.4) years, mean BMI of (22.2 ± 3.5) kg/m<sup>2</sup>, median age-adjusted Charlson comorbidity index (aCCI) of 1.0 (0.0, 1.0), and preoperative creatinine level of (75.3±15.9) μmol/L; tumors were located in the left kidney in 7 patients (53.8%) and right kidney in 6 (46.2%); preoperative distant metastasis (M1 stage) was present in 6 patients (46.2%), while 7 (53.8%) showed no metastasis; VT distribution by Mayo level comprised 7 cases (53.8%) at level 0, 1 case each at levels Ⅰ and Ⅳ (7.7% respectively), and 2 cases each at levels Ⅱ and Ⅲ (15.4% respectively); surgical approaches comprised open surgery (<i>n</i>=2, 15.4%), laparoscopic surgery (<i>n</i>=6, 46.1%), and robot-assisted laparoscopic surgery (<i>n</i>=5, 38.5%); mean operative time was (273±79) min, and intraoperative blood loss was (722±570) mL; mean maximum tumor diameter was (10.8±2.4) cm. All the 13 patients underwent TFE3 protein immunohistochemistry (IHC) staining, with 7 confirmed by fluorescence <i>in situ</i> hybridization (FISH). Tumor recurrence or metastasis occurred in 11 patients (84.6%), and 9 (69.2%) patients died during follow-up. Median PFS was 4 months (1 year PFS rate: 31%), and median OS was 13 months (1 year OS rate: 54%). Survival analysis of 45 <i>TFE3</i>-RCC patients revealed statistically significant differences in PFS and OS across all the clinical stages (<i>P</i> < 0.001). The <i>TFE3</i>-VT patients exhibited significantly worse PFS and OS than the <i>TFE3</i>-non-VT patients (<i>P</i> < 0.001), with persistent significance in stage Ⅲ subgroup analysis (<i>P</i> < 0.05). After PSM, <i>TFE3</i>-VT patients showed significantly inferior PFS compared with non-<i>TFE3</i>-VT (<i>P</i>=0.01), though no significant difference was shown between the OS curves (<i>P</i>=0.11).</p><p><strong>Conclusion: </strong><i>TFE3</i>-VT predominantly occurs in young females with frequent preoperative metastases. Strongly-positive staining of TFE3 protein in IHC staining and red-green split signals in FISH tests are reliable diagnostic markers. <i>TFE3</i>-VT patients exhibit inferior survival compared with <i>TFE3</i>-non-VT patients and earlier progression than non-<i>TFE3</i>-VT patients.</p>\",\"PeriodicalId\":8790,\"journal\":{\"name\":\"北京大学学报（医学版）\",\"volume\":\"57 4\",\"pages\":\"650-661\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-08-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12340522/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"北京大学学报（医学版）\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"北京大学学报（医学版）","FirstCategoryId":"3","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
[Clinicopathological features and survival analysis of TFE3-rearranged renal cell carcinoma with venous tumor thrombus].
Objective: To review the clinicopathological features of TFE3-rearranged renal cell carcinoma (TFE3-RCC) with venous tumor thrombus (VT) (TFE3-VT), to explore treatment strategies and to prognostic characteristics, and to provide diagnostic and therapeutic references for TFE3-VT patients.
Methods: Patients who underwent surgery at Department of Urology, Peking University Third Hospital from January 2013 to January 2024 were enrolled, including three cohorts: Pathologically confirmed TFE3-VT patients, TFE3-RCC patients without VT (TFE3-non-VT), and non-TFE3-rearranged renal cell carcinoma patients with VT (non-TFE3-VT). Clinical history, imaging data, pathological data, and follow-up records were collected. Primary and secondary endpoints were progression-free survival (PFS) and overall survival (OS), respectively. (1) Baseline characteristics were compared between the TFE3-VT and TFE3-non-VT patients. Normally distributed continuous variables were expressed as mean±SD and compared using Student's t-test; non-normally distributed variables were expressed as M (P25, P75) and analyzed with Mann-Whitney U test; categorical variables were described as frequency and percentage [n (%)] and compared by χ2 test or Fisher's exact test. (2) Clinical history, radiological presentations, surgical data, and histopathological features of the TFE3-VT patients were comprehensively characterized. (3) Survival analysis was performed for the TFE3-VT patients. Follow-up data of the TFE3-VT patients were described in detail, and their survival outcomes were compared with the TFE3-non-VT and non-TFE3-VT patients. When compared with the TFE3-non-VT counterparts, Kaplan-Meier method was used to generate PFS and OS curves among: (1) the TFE3-RCC patients across clinical stages Ⅰ-Ⅳ; (2) TFE3-VT versusTFE3-non-VT cohorts; (3) stage Ⅲ subgroups of the TFE3-VT and TFE3-non-VT patients. Intergroup survival differences were statistically evaluated using Log-rank tests. For comparisons with the non-TFE3-VT patients, a 1 : 1 propensity score matching (PSM) was implemented to balance baseline characteristics between the two cohorts. Post-matching Kaplan-Meier curves were generated to compare PFS and OS between the matched groups, with Log-rank tests employed to determine statistical significance of survival disparities. All statistical analyses were conducted with R software (v 4.2.3), and two-tailed P < 0.05 was considered statistically significant.
Results: The study included 45 TFE3-RCC patients: 13 TFE3-VT and 32 TFE3-non-VT cases. Additionally, 523 non-TFE3-VT patients were enrolled. Among the 13 TFE3-VT patients, 9 were female (69.2%) and 4 male (30.8%), with a mean age of (37.9±14.4) years, mean BMI of (22.2 ± 3.5) kg/m2, median age-adjusted Charlson comorbidity index (aCCI) of 1.0 (0.0, 1.0), and preoperative creatinine level of (75.3±15.9) μmol/L; tumors were located in the left kidney in 7 patients (53.8%) and right kidney in 6 (46.2%); preoperative distant metastasis (M1 stage) was present in 6 patients (46.2%), while 7 (53.8%) showed no metastasis; VT distribution by Mayo level comprised 7 cases (53.8%) at level 0, 1 case each at levels Ⅰ and Ⅳ (7.7% respectively), and 2 cases each at levels Ⅱ and Ⅲ (15.4% respectively); surgical approaches comprised open surgery (n=2, 15.4%), laparoscopic surgery (n=6, 46.1%), and robot-assisted laparoscopic surgery (n=5, 38.5%); mean operative time was (273±79) min, and intraoperative blood loss was (722±570) mL; mean maximum tumor diameter was (10.8±2.4) cm. All the 13 patients underwent TFE3 protein immunohistochemistry (IHC) staining, with 7 confirmed by fluorescence in situ hybridization (FISH). Tumor recurrence or metastasis occurred in 11 patients (84.6%), and 9 (69.2%) patients died during follow-up. Median PFS was 4 months (1 year PFS rate: 31%), and median OS was 13 months (1 year OS rate: 54%). Survival analysis of 45 TFE3-RCC patients revealed statistically significant differences in PFS and OS across all the clinical stages (P < 0.001). The TFE3-VT patients exhibited significantly worse PFS and OS than the TFE3-non-VT patients (P < 0.001), with persistent significance in stage Ⅲ subgroup analysis (P < 0.05). After PSM, TFE3-VT patients showed significantly inferior PFS compared with non-TFE3-VT (P=0.01), though no significant difference was shown between the OS curves (P=0.11).
Conclusion: TFE3-VT predominantly occurs in young females with frequent preoperative metastases. Strongly-positive staining of TFE3 protein in IHC staining and red-green split signals in FISH tests are reliable diagnostic markers. TFE3-VT patients exhibit inferior survival compared with TFE3-non-VT patients and earlier progression than non-TFE3-VT patients.
期刊介绍：
Beijing Da Xue Xue Bao Yi Xue Ban / Journal of Peking University (Health Sciences), established in 1959, is a national academic journal sponsored by Peking University, and its former name is Journal of Beijing Medical University. The coverage of the Journal includes basic medical sciences, clinical medicine, oral medicine, surgery, public health and epidemiology, pharmacology and pharmacy. Over the last few years, the Journal has published articles and reports covering major topics in the different special issues (e.g. research on disease genome, theory of drug withdrawal, mechanism and prevention of cardiovascular and cerebrovascular diseases, stomatology, orthopaedic, public health, urology and reproductive medicine). All the topics involve latest advances in medical sciences, hot topics in specific specialties, and prevention and treatment of major diseases.
The Journal has been indexed and abstracted by PubMed Central (PMC), MEDLINE/PubMed, EBSCO, Embase, Scopus, Chemical Abstracts (CA), Western Pacific Region Index Medicus (WPR), JSTChina, and almost all the Chinese sciences and technical index systems, including Chinese Science and Technology Paper Citation Database (CSTPCD), Chinese Science Citation Database (CSCD), China BioMedical Bibliographic Database (CBM), CMCI, Chinese Biological Abstracts, China National Academic Magazine Data-Base (CNKI), Wanfang Data (ChinaInfo), etc.
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