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1.
PurposeTo investigate the safety and efficacy of an aqueous polyethylene glycol-based liquid embolic agent, Embrace Hydrogel Embolic System (HES), in the treatment of benign and malignant hypervascular tumors.Materials and MethodsA prospective, single-arm, multicenter study included 8 patients, 5 males and 3 females, with a median age of 58.5 years (30–85 years), who underwent embolization in 8 tumors between October 2019 and May 2020. Technical success was defined as successful delivery of HES to the index vessel, with disappearance of >90% of the targeted vascular enhancement or, for portal vein embolization, occlusion of the portal branches to the liver segments for future resection. The volume of HES administered, ease of use (5 point Likert scale), administration time, and adverse events (AEs) were recorded. Evaluation was performed at 7, 30, and 90 days via clinical assessment and blood testing, and follow-up imaging was performed at 30 days.ResultsEight patients were enrolled, and 10 embolizations were performed in 8 lesions. Tumors included hepatocellular carcinoma (n = 4), renal angiomyolipoma (n = 3), and intrahepatic cholangiocarcinoma (n = 1). Technical success was 100%, and the average ease of use was 3.3 ± 1.0 SD. The HES delivery time was 1–28 minutes (median, 16.5 minutes), and the HES volume injected was 0.4–4.0 mL (median, 1.3 mL). All patients reached 30-day follow-up with imaging, and 6 patients reached 90-day follow-up. There were 3 serious AEs in 2 patients that were unrelated to the embolic agent.ConclusionHES resulted in a 100% embolization technical success rate. The product ease of use was acceptable, and no target vessel recanalization was noted on follow-up imaging at 30 days.  相似文献   
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ObjectiveTo study the clinical features and identify unique renal neoplasia subtypes and their prognostic implications in individuals with tuberous sclerosis complex (TSC).Patients and MethodsThe Mayo Clinic nephrectomy registry included 37 patients with TSC diagnosed between 1970 and 2018. Four additional patients were identified from the pathology consultation and autopsy files. All available renal tumors were further characterized using immunohistochemistry and fluorescence in situ hybridization. Clinicopathologic features and follow-up were obtained from the medical record. The American Association for Cancer Research Project GENIE registry was accessed using cBioPortal for molecular profiling of angiomyolipoma (AML).ResultsA total of 276 renal tumors from 41 patients were analyzed. Renal tumors were classified into 9 distinct morphological subtypes, with AML predominating (238 [86%]). Interestingly, all these tumors acted in a benign fashion except one renal cell carcinoma with clear cells and fibromyomatous stroma and one epithelioid AML that metastasized. Molecular profiling studies revealed that epithelioid AMLs were enriched for alterations of TP53, RB1, and ATRX. Eight patients died of direct complications of TSC, including 3 of end-stage renal disease. To date, none have died of a renal epithelial neoplasm.ConclusionThe identification of unique renal neoplasia subtypes may provide important clues to establish a diagnosis of TSC, and in the somatic setting, this finding has important implications for accurate prognostication. These tumors tend to be indolent, and only 2 of 276 tumors in our study exhibited metastatic behavior. Our results support multidisciplinary management with a focus on preservation of renal function.  相似文献   
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目的对比研究腹腔镜下外生性肾血管平滑肌脂肪瘤"蘑菇状"剜除与标准肾部分切除术的安全性及有效性,为肾血管平滑肌脂肪瘤腹腔镜下"蘑菇状"剜除术术式的建立提供临床依据。 方法选取海南医学院第二附属医院与解放军总医院2018年1月至2019年5月期间,接受腹腔镜手术的肾血管平滑肌脂肪瘤患者53例,其中25例沿肿瘤假包膜行"蘑菇状"剜除术(A组)、28例行肾部分切除术(B组)。比较两组手术患者的肾动脉阻断时间、手术时间、术中出血量、术后24 h血红蛋白、术后eGFR(estimated glomerular filtration rate,估计肾小球率过滤)的变化、术后住院时间和术后肿瘤复发率。 结果53例手术无术中转开放,无死亡病例。肾动脉阻断时间:A组(11.9±2.2)min、B组(21.5±6.5) min(P<0.001)。手术时间:A组(87.9±24.8)min、B组(114.3±38.9) min(P<0.001)。术中出血量:A组20 ml(20~40)ml、B组50 ml(50~100)ml(P<0.001)。术后24 h血红蛋白变化:A组(7.4±4.3) g/L、B组(12.4±8.8) g/L(P=0.013)。术后24 h eGFR变化:A组(6.2±7.2 )ml(min·1.73 m2),B组(12.7±12.8)ml(min·1.73 m2)(P=0.027)。术后6个月eGFR变化:A组(1.5±3.7)ml(min·1.73 m2)、B组(6.5±5.6)ml(min·1.73 m2)(P<0.001)。术后住院时间:A组4.0 d(3~4)d、B组4.5 d(3~6)d(P=0.023)。术后随访两组术后肿瘤均无复发。 结论采用腹腔镜"蘑菇状"剜除术治疗外生性肾血管平滑肌脂肪瘤在肾动脉阻断时间、术中出血量、术后24 h血红蛋白、术后eGFR变化、术后住院时间等方面均优于传统肾部分切除术,两组术后肿瘤均无复发;该方法安全、有效,适于临床推广。  相似文献   
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Twenty-five pathologically proven hepatic angiomyolipomas (AMLs) were included in the study. Ultrasonic features of hepatic AMLs were reviewed. Three types of echogenicity were observed on ultrasound examination: (i) strong hyper-echogenicity, (ii) moderate hyper-echogenicity and (iii) hypo-echogenicity. Vascular signals within tumors could be detected in 22 (88.00%) tumors as multiple punctiform, filiform or dendriform signals by color Doppler flow imaging. Based on the enhancement patterns in the arterial, portal and late phases, the features of hepatic AMLs on contrast-enhanced ultrasound were divided into four subtypes: (i) “fast in slow out” (68.00%, n = 17); (ii) “fast in same out” (16%, n = 4); (iii) “fast in fast out” (12.00%, n = 3); and (iv) “fast in uneven out” (4.00%, n = 1). Contrast-enhanced ultrasound diagnosed 22 (88.00%) tumors as benign tumors and 13 (52.00%) as hepatic AMLs. Four cases were misdiagnosed as hepatic hemangioma, five cases as focal nodular hyperplasia (total = 36.00%). The rate of correct diagnosis of hepatic AMLs increased significantly from 24.00% for ultrasound alone to 52.00% for contrast-enhanced ultrasound. Therefore, information obtained from ultrasound, color Doppler flow imaging and contrast-enhanced ultrasound should be combined to improve diagnosis.  相似文献   
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BackgroundPartial nephrectomy or angioembolisation is commonly used for sporadic renal angiomyolipomas (RAMLs) with high RENAL scores, but there is a risk of reduced renal function, postoperative complications, and recurrence.ObjectiveTo describe a new technique for off-clamp laparoscopic evacuation of sporadic RAMLs with high RENAL scores that promotes maximal renal function maintenance and low postoperative complication and lesion recurrence rates.Design, setting, and participantsA retrospective cohort of patients undergoing off-clamp laparoscopic evacuation for sporadic RAMLs with RENAL scores ≥9 from January 2013 to June 2018 was included.Surgical procedureWe highlighted the curettage, suction, packing, and binding (CSPB) technique, a new off-clamp retroperitoneoscopic evacuation technique for sporadic RAMLs.MeasurementsDemographics, preoperative, intraoperative, and postoperative outcomes were assessed.Results and limitationsA total of 141 cases were included. The median (interquartile range [IQR]) tumour size was 7 (6.2–8.2) cm. The median (IQR) RENAL score was 10 (9–11). The median (IQR) operative time was 80 (65–125) min, with a median (IQR) estimated blood loss of 130 (90–362.5) ml. Conversion to neither open surgery nor standard laparoscopy occurred. The warm ischaemia time was zero for all cases. Postoperatively, 13 minor complications (Clavien grade 1) were recorded. No blood transfusions were reported. The glomerular filtration rate did not change significantly from preoperative period to 12-mo follow-up. Recurrence did not occur at the median follow-up period of 48 (36–60) mo. The retrospective design and lack of a control group are limitations of this study.ConclusionsOff-clamp retroperitoneoscopic tumour evacuation using the CSPB technique is feasible, safe, and effective for treating complex sporadic RAMLs.Patient summaryWe report a curettage, suction, packing, and binding technique for off-clamp retroperitoneoscopic evacuation of sporadic renal angiomyolipomas that leads to complete lesion clearance, excellent renal function preservation, and minimal perioperative complications.  相似文献   
9.
Renal epithelioid angiomyolipoma (EAML) is a potentially malignant tumor type whose characteristics and biomarkers predictive of malignant behavior have not been elucidated. Here, we report three cases of renal EAML with malignant features but without histories of tuberous sclerosis complex. Case 1 involved a 29‐year‐old man with a 12‐cm solid mass in the right kidney who underwent radical right nephrectomy. Case 2 involved a 22‐year‐old woman with a retroperitoneal mass who underwent radical right nephrectomy and retroperitoneal tumorectomy. Local recurrence was detected 7 years post‐surgery. Case 3 involved a 23‐year‐old man with a 14‐cm solid mass in the left kidney who underwent radical left nephrectomy. Microscopically, the tumors in all cases demonstrated proliferation of epithelioid cells with atypia, mitotic activity, necrosis, hemorrhage, and vascular invasion. Epithelioid cells in all cases were immunohistochemically positive for melanocytic and myoid markers and weakly positive for E‐cadherin and β‐catenin. Immunohistochemistry revealed activation of the mammalian target of rapamycin pathway. Here, we report the morphological and immunohistochemical features of clinically or histologically malignant renal EAML.  相似文献   
10.
目的 研究基于随机投影的多分类器分层融合的分类模型对良性肾小肿块乏脂肪肾血管平滑肌脂肪瘤(<4 cm)(AMLwvf)和恶性肾小肿块肾细胞癌(RCC)的鉴别能力。方法 回顾性收集163例经病理证实存在肾小肿块的患者,其中118例为肾细胞癌,45例为乏脂肪肾血管平滑肌脂肪瘤,对平扫CT图像中病灶面积最大的代表性切片进行目标感兴趣区域(ROI)勾画,利用放射组学特征构建一个层次型的融合框架。在投影域水平上对同质分类器进行融合,然后在分类器水平上对融合结果进行进一步融合,最终得到基于随机投影的多分类器分层融合的AMLwvf和RCC鉴别分类模型。采用五折交叉验证方法和特异性(SPE)、灵敏度(SEN)、准确率(ACC)、ROC曲线下面积(AUC)评价AMLwvf与RCC鉴别分类模型的性能。将本研究所提模型与使用单一基分类器算法以及几种传统的集成模型对AMLwvf和RCC的鉴别分类能力进行定量比较,验证本研究所提鉴别模型的可行性和有效性。结果 投影数设置为10时,本文提出的分层融合鉴别模型在所有指标上获得最好的结果。基于投影数为10的前提,五折交叉验证结果显示本研究所提出的基于多分类器分层融合的AMLwvf和RCC鉴别分类模型的SPE、SEN、ACC、AUC分别为:0.853、0.693、0.809、0.870。结论 基于随机投影的多分类器集成分类系统构建的AMLwvf和RCC鉴别模型可以很好地对 AMLwvf 和 RCC 进行鉴别分类。同时与基于单一分类器算法以及其他多分类器集成系统构建的AMLwvf和RCC的鉴别模型相比,本文所提出鉴别模型在AMLwvf和RCC的鉴别分类任务中具有较大优势。  相似文献   
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