A Pilot First-in-Human Study of Embrace,a Polyethylene Glycol-Based Liquid Embolic Agent,in the Embolization of Malignant and Benign Hypervascular Tumors

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.     

腹腔镜"蘑菇状"剜除术治疗外生性肾血管平滑肌脂肪瘤的临床应用

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。     

肾血管平滑肌脂肪瘤23例并文献复习

目的探讨肾血管平滑肌脂肪瘤的诊治方法.方法回顾性分析23例肾血管平滑肌脂肪瘤患者的临床资料并结合文献讨论其诊断与治疗问题.结果术前误诊为肾癌3例,23例均行不同手术治疗,术后病理证实为肾血管平滑肌脂肪瘤,术前选择性肾动脉栓塞或术中低温下阻断肾蒂可显著减少出血和不必要的肾切除.结论B超、CT是诊断本病的主要检查方法,彩超具有较好的良性疾病提示率,术中诊断不明者行多点取材冰冻切片具有重要意义,手术原则是尽可能保留肾单位.     

Hepatic angiomyolipoma developing during the follow-up of ulcerative colitis: Report of a case and review of the literature

Hepatic angiomyolipoma is a rare tumor composed of spindle-shaped and epithelioid smooth muscle cells, adipose tissue, and proliferating blood vessels. We report the first documented case of this tumor developing in a patient with ulcerative colitis. A solitary tumor (7.5×7.5×7cm) was detected in the left lateral segment of the liver and a left hepatic lobectomy was performed. The diagnosis of angiomyolipoma was confirmed by a pathological examination. We also review the literature on previously reported cases of hepatic angiomyolipoma.     

Angiomyolipoma mimicking true lipoma of the liver: report of two cases

Hepatic angiomyolipoma (AML) is very rare and only about 80 cases have been reported. The tumor is fundamentally heterogeneously composed of the three tissue components of blood vessels, smooth muscle cells (SMC), and fat cells. Two cases of hepatic AML are reported here, both of which are histologically composed predominantly of a fat cell element and resembled true lipoma (lipomatous AML). However, careful examination of both tumors revealed the presence of a small amount of epithelioid SMC, especially around blood vessels. Immunohistochemical study using monoclonal antibody for melanoma (HMB-45) clearly revealed a small amount of HMB-45-positive SMC around the blood vessels and scattered in the diffuse fat cell growth in both tumors. Since no liver tissue components or primary liver tumors are reactive with HMB-45 except AML cells, the presence of HMB-45-positive cells within the tumor clearly established the diagnosis of hepatic AML. Any fatty tumor or focal fatty lesion of the liver that superficially resemble true lipomas should be tested for the presence of HMB-45-positive SMC in the tumor to differentiate it from AML.     

Hepatic angiomyolipoma and hepatic stellate cells share a similar gene expression profile

BACKGROUND AND AIMS: Angiomyolipomas (AMLs) of the liver are rare neoplasms composed of large epithelioid cells with intermixed fat and blood vessels. Hepatic AMLs have no clear normal-cell counterpart in the liver. However, AMLs and stellate cells both are positive for neural crest-derived markers including HMB-45 antigen. METHODS: To further explore the similarities between hepatic AMLs and stellate cells, gene expression of a hepatic AML was studied by cDNA microarray. Real-time polymerase chain reaction was used to confirm gene expression. Hepatic stellate cells can be quiescent, activated, or have a myofibroblastic phenotype depending on their state of activation. Expression of known markers of activated stellate cells was compared between the AML, activated primary mouse stellate cells, and stellate cell lines with activated and myofibroblastic phenotypes. Next, 5 novel genes from the AML were selected because they were not previously known to be markers of stellate cells and mRNA expression measured in the activated mouse stellate cells and in myofibroblastic stellate cell lines. Finally, expression levels of 10 novel genes were determined in 5 cirrhotic and 5 noncirrhotic human livers. RESULTS: Overexpression of known markers of activated stellate cells including transforming growth factor beta (TGF- beta ), smooth muscle actin, and collagen was found in the hepatic AML. Three of 5 novel markers that were identified in the AML, RRAD (Ras-related associated with diabetes), CTSK (cathepsin K), and NIBAN were also found to be overexpressed in activated stellate cells compared with quiescent or myofibroblastic stellate cells. In addition, 9 of 10 novel genes overexpressed in AML were also overexpressed in cirrhotic human livers versus noncirrhotic livers. CONCLUSIONS: Hepatic AMLs share a similar gene expression profile and may differentiate toward activated stellate cells.     

Triple synchronous neoplasms in one kidney: report of a case and review of the literature

We report the case of a patient with three synchronous but histologically different primary renal tumors that were all in the same kidney. Two tumors were different subtypes of renal cell carcinoma (RCC), and the third was a variant form of angiomyolipoma. The patient was a 62-year-old man who was receiving antihypertensive drugs and came to our hospital for a regular check-up. Ultrasonography performed during the visit revealed a left renal mass, but the patient had no related symptoms. Subsequent computed tomography revealed two round, high-density masses, one in the mid-portion and the other in the lower pole of the left kidney, and multiple cysts in the right kidney and the liver. The mass in the mid-portion measured 3.3 x 3.0 x 2.8 cm, and the mass in the lower pole measured 1.7 x 1.1 x 0.9 cm. A left radical nephrectomy was performed. On gross examination, an additional ovoid nodule (0.6 cm in the greatest dimension) was found in the lower pole. Microscopically, the largest tumor consisted of a broad alveolar arrangement of large round cells with abundant eosinophilic or clear cytoplasm, distinct cell borders, and perinuclear halos, features consistent with chromophobe RCC. The smallest tumor was a conventional (clear-cell) RCC. The third tumor was composed solely of atypical epithelioid cells with prominent nucleoli and yellowish-brown to black pigments. The tumor cells were positive for melanin (Fontana-Masson stain), the melanoma marker HMB45, vimentin, smooth-muscle actin, and the macrophage marker CD68 and were negative for cytokeratin. This tumor was considered a pigmented epithelioid type of angiomyolipoma. The histologic, histochemical, and immunohistochemical features in this case confirmed the presence of three synchronous primary tumors, a chromophobe and a clear-cell type RCC and a pigmented epithelioid angiomyolipoma, all of which were in the same kidney. This case is the first of its type reported in the literature.     

Off-clamp Retroperitoneoscopic Tumour Evacuation for Sporadic Renal Angiomyolipomas with High RENAL Nephrometry Scores: A Novel Surgical Technique and Its Outcomes

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.     

45例肾血管肌脂瘤的治疗

目的：探讨肾血管肌脂瘤的治疗方法。方法：４５例患者中２３例未作特殊治疗;６例超选择性动脉栓塞治疗、７例行肿瘤剜除术或肾部分切除术、９例行全肾切除术。结果：随访２１例,平均随访时间３．６ａ。未作特殊治疗的病例中未发现肿瘤破裂出血,２便肿瘤体积增大但我症状;超选择性动脉栓塞病例无症状复发、无破裂出血、肾功能稳定。手术患者均保持肾功能稳定,无肿瘤复发。结论：对本病采取保守性治疗原则。超选择肾动脉栓塞是简