Cysts

囊肿
  • 文章类型: Journal Article
    肝脏局灶性病变(FLL)已成为腹部成像中越来越常见的发现,尤其是无症状和偶然的肝脏病变。胃肠病学家和肝病学家经常在咨询中看到这些患者,并为多种类型的肝脏病变的管理提出建议,包括肝细胞腺瘤,局灶性结节增生,血管瘤,和肝囊性病变,包括多囊性肝病。恶性肿瘤在FLL的鉴别诊断中很重要,医疗保健提供者必须熟悉FLL的诊断和管理。该美国胃肠病学实践指南使用可用的最佳证据为最常见的FLL做出诊断和管理建议。
    Focal liver lesions (FLLs) have become an increasingly common finding on abdominal imaging, especially asymptomatic and incidental liver lesions. Gastroenterologists and hepatologists often see these patients in consultation and make recommendations for management of multiple types of liver lesions, including hepatocellular adenoma, focal nodular hyperplasia, hemangioma, and hepatic cystic lesions including polycystic liver disease. Malignancy is important to consider in the differential diagnosis of FLLs, and healthcare providers must be familiar with the diagnosis and management of FLLs. This American College of Gastroenterology practice guideline uses the best evidence available to make diagnosis and management recommendations for the most common FLLs.
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  • 文章类型: Journal Article
    结肠重复构成一种罕见的先天性异常,特征在于存在表现出上皮衬里的肠壁的中空囊性或管状结构。由于其发病率低和表现为腹痛或便秘等非特异性症状,诊断挑战持续存在。导致不愿进行手术切除。由于结肠重复中的相关恶性肿瘤很少见,这些异常的固有恶性潜力仍未确定。此外,尽管有结肠重复相关恶性肿瘤的报道,文献中没有关于这些病例中的管状腺瘤的详细报道.该病例的组织学特征特别值得注意,位于癌前阶段,在结肠重复内潜在的腺癌进展。
    Colonic duplication constitutes a rare congenital anomaly, characterized by the presence of hollow cystic or tubular structures exhibiting an epithelial-lined intestinal wall. Diagnostic challenges persist due to its low incidence and manifestation of nonspecific symptoms such as abdominal pain or constipation, resulting in a reluctance to pursue surgical resection. As associated malignancies in colonic duplication are rare, the inherent malignant potential of these anomalies remains undetermined. Additionally, despite reported instances of associated malignancies in colonic duplication, there is an absence of reports in the literature detailing tubular adenoma within these cases. The histologic features of the presented case are particularly noteworthy, situated at the precancerous stage, intimating potential progression towards adenocarcinoma within colonic duplication.
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  • 文章类型: Journal Article
    胆道囊肿(PC)是壁外胆管腺体的扩张,在肝胆疾病的背景下具有非常独特的成像模式,特发性门静脉高压症,成人肝脏和肾脏多囊疾病,单发非寄生虫囊肿,和全身性感染。PC的临床相关性与以下事实有关:它们的存在可能表明潜在的病理(例如上面提到的那些),并且可以被认为是肝病进展的潜在标志物。虽然影像学检查结果很有特点,认识到他们的主要鉴别诊断,包括恶性肿瘤,可能具有挑战性,但对于避免诊断错误至关重要。
    Peribiliary cysts (PC) are dilatations of the extramural peribiliary glands, with a very characteristic imaging pattern in the contexts of hepatobiliary diseases, idiopathic portal hypertension, adult-type polycystic disease of the liver and kidneys, solitary nonparasitic cysts, and systemic infections. The clinical relevance of PC is related to the fact that their presence may indicate underlying pathologies (such as those mentioned above) and may be considered as a potential marker of liver disease progression. Although imaging findings are quite characteristic, recognizing their main differential diagnoses, including malignancies, can be challenging but are essential to avoiding diagnostic errors.
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  • 文章类型: English Abstract
    这项研究的目的是探讨产前磁共振成像(MRI)在评估胎儿先天性囊性肺病中的实用价值,评估病变的相对大小和肺发育状况,并尝试在后处理中利用MRI的强度来获得病变大小和肺部发育状况的评估指标,可以预测这些胎儿出生后可能面临的预后。我们回顾性收集并分析了诊断为先天性囊性肺病的胎儿数据。这些胎儿的产前超声检查导致诊断他们怀疑患有先天性囊性肺病,并且随后的产前MRI证实了诊断。对胎儿进行随访以跟踪其出生时的状况(产后呼吸窘迫,机械通气,等。),胎儿是否接受了手术治疗,以及手术治疗后胎儿的恢复。随访胎儿的恢复情况,探讨产前MRI检查评估胎儿先天性肺囊性疾病的可行性,初步探讨产前MRI对先天性肺囊性疾病胎儿预后的预测价值。
    MRI胎儿图像收集自2018年5月至2023年3月在四川大学华西第二医院就诊的孕妇,通过产前超声和随后的MRI诊断为胎儿先天性肺囊性疾病。对先天性囊性肺病胎儿MRI图像进行后处理,获得胎儿肺部病变体积,胎儿受累的肺容积,健康的肺容量,和胎儿头围测量。肺和肝脏的信号强度,病变体积/受影响的肺体积,病变体积/总肺体积,囊性体积比(CVR),并测量双侧肺-肝信号强度比。结合胎儿出生后6个月的随访结果,进一步分析MRI后处理采集指标对先天性囊性肺病胎儿预后评估的可行性和价值。采用Logistic回归模型对产妇年龄、MRI时的孕周,CVR,和双侧肺-肝信号强度比,并评估这些指标是否与不良预后相关。受试者工作特征(ROC)曲线用于评估单独通过MRI计算获得的参数以及与预测出生后不良预后的多种指标相结合的参数的价值。
    我们收集了2018年5月至2023年3月间通过胎儿MRI诊断为先天性囊性肺病的胎儿共67例,并排除了6例受影响肺部无正常肺组织的病例,11例胎儿诱导,3例失孕。最后,纳入47例先天性囊性肺病胎儿,其中30例预后良好,17例预后不良。预后良好组与预后不良组胎儿患侧和健侧肺、肝的信号强度比值差异有统计学意义(P<0.05),健康侧肺和肝脏的信号强度比高于患侧肺和肝脏的信号强度比。进一步分析表明,CVR(比值比[OR]=1.058,95%置信区间[CI]:1.014-1.104),患侧和健康侧的肺-肝信号强度比之间的差异(OR=0.814,95%CI:0.700-0.947)与先天性囊性肺病胎儿的出生预后不良相关。此外,ROC曲线分析显示,联合应用病变体积/受累肺体积和观察到的受累肺与健康肺和肝脏之间信号强度比值的差异,比单参数判断更准确地预测先天性囊性肺病患儿的预后。曲线下面积为0.988,截止值为0.33,对应灵敏度为100%,特异性为93.3%,95%CI为0.966-1.000。
    基于先天性囊性肺病胎儿的MRI,我们获得了病变体积的信息,病变体积/受影响的肺体积,病变体积/总肺体积,CVR,和双侧肺-肝信号强度比值差异,所有这些都在预测先天性囊性肺病胎儿的不良预后方面显示出一定的临床价值。此外,在组合指数中,病变体积/受累肺体积和双侧肺-肝信号强度比差异是先天性囊性肺病胎儿预后不良的更有效预测因子。在预测先天性囊性肺病胎儿的不良预后方面有较好的疗效。这为进一步评估先天性囊性肺病胎儿的肺发育提供了一种新的有效的预测方法。有助于提高对先天性囊性肺病胎儿预后的评估和预测。
    UNASSIGNED: The aim of this study is to explore the practical value of prenatal magnetic resonance imaging (MRI) in the assessment of congenital cystic lung disease in fetuses, to evaluate the relative size of the lesion and the status of lung development, and to make an attempt at utilizing the strength of MRI in post-processing to obtain assessment indicators of the size of the lesion and the status of lung development, with which predictions can be made for the prognosis that these fetuses may face after birth. We retrospectively collected and analyzed the data of fetuses diagnosed with congenital cystic lung disease. Prenatal ultrasound examination of these fetuses led to the diagnosis that they were suspected of having congenital cystic lung disease and the diagnosis was confirmed by subsequent prenatal MRI. The fetuses were followed up to track their condition at birth (postnatal respiratory distress, mechanical ventilation, etc.), whether the fetuses underwent surgical treatment, and the recovery of the fetuses after surgical treatment. The recovery of the fetuses was followed up to explore the feasibility of prenatal MRI examination to assess fetal congenital pulmonary cystic disease, and to preliminarily explore the predictive value of prenatal MRI for the prognosis of fetuses with congenital pulmonary cystic disease.
    UNASSIGNED: MRI fetal images were collected from pregnant women who attended the West China Second University Hospital of Sichuan University between May 2018 and March 2023 and who were diagnosed with fetal congenital pulmonary cystic disease by prenatal ultrasound and subsequent MRI. Fetal MRI images of congenital cystic lung disease were post-processed to obtain the fetal lung lesion volume, the fetal affected lung volume, the healthy lung volume, and the fetal head circumference measurements. The signal intensity of both lungs and livers, the lesion volume/the affected lung volume, the lesion volume/total lung volume, the cystic volume ratio (CVR), and the bilateral lung-liver signal intensity ratio were measured. The feasibility and value of MRI post-processing acquisition indexes for evaluating the prognosis of fetuses with congenital cystic lung disease were further analyzed by combining the follow-up results obtained 6 months after the birth of the fetus. Logistic regression models were used to quantify the differences in maternal age, gestational week at the time of MRI, CVR, and bilateral lung-to-liver signal intensity ratio, and to assess whether these metrics correlate with poor prognosis. Receiver operating characteristic (ROC) curves were used to assess the value of the parameters obtained by MRI calculations alone and in combination with multiple metrics for predicting poor prognosis after birth.
    UNASSIGNED: We collected a total of 67 cases of fetuses diagnosed with congenital cystic lung disease by fetal MRI between May 2018 and March 2023, and excluded 6 cases with no normal lung tissue in the affected lungs, 11 cases of fetal induction, and 3 cases of loss of pregnancy. In the end, 47 cases of fetuses with congenital cystic lung disease were included, of which 30 cases had a good prognosis and 17 cases had a poor prognosis. The difference in the difference between the signal intensity ratios of the affected and healthy sides of the lungs and livers of the fetuses in the good prognosis group and that in the poor prognosis group was statistically significant (P<0.05), and the signal intensity ratio of the healthy side of the lungs and livers was higher than the signal intensity ratio of the affected side of the lungs and livers. Further analysis showed that CVR (odds ratio [OR]=1.058, 95% confidence interval [CI]: 1.014-1.104), and the difference between the lung-to-liver signal intensity ratios of the affected and healthy sides (OR=0.814, 95% CI: 0.700-0.947) were correlated with poor prognosis of birth in fetuses with congenital cystic lung disease. In addition, ROC curve analysis showed that the combined application of lesion volume/affected lung volume and the observed difference in the signal intensity ratio between the affected and healthy lungs and liver predicted the prognosis of children with congenital cystic lung disease more accurately than the single-parameter judgment did, with the area under the curve being 0.988, and the cut-off value being 0.33, which corresponded to a sensitivity of 100%, a specificity of 93.3%, and a 95% CI of 0.966-1.000.
    UNASSIGNED: Based on the MRI of fetuses with congenital cystic lung disease, we obtained information on lesion volume, lesion volume/affected lung volume, lesion volume/total lung volume, CVR, and bilateral lung-to-liver signal intensity ratio difference, all of which showing some clinical value in predicting the poor prognosis in fetuses with congenital cystic lung disease. Furthermore, among the combined indexes, the lesion volume/affected lung volume and bilateral lung-to-liver signal intensity ratio difference are more effective predictors for the poor prognosis of fetuses with congenital cystic lung disease, and show better efficacy in predicting the poor prognosis of fetuses with congenital cystic lung disease. This provides a new and effective predictive method for further assessment of pulmonary lung development in fetuses with congenital cystic lung disease, and helps improve the assessment and prediction of the prognosis of fetuses with congenital cystic lung disease.
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  • 文章类型: Journal Article
    尽管在伊比利亚半岛海岸报告的第一次赤潮是由于多林格洛丁,了解他们的频率和,特别是,关于促成开花的环境条件仍然很少。出于这个原因,根据1993-2008年记录数据库期间,在夏季的三个加利西亚rias中观察和研究了L.polyedra开花事件;此外,样本于2008年夏季收集。6月和8月,在阿瑞斯和巴奎罗的rias中发生了Polyedra的增殖,分别,在科鲁尼亚的Ria,他们从六月底坚持到九月初。当地表温度达到17℃时,出现了赤潮,具有“季节性热窗”条件,当盐度≥30时,即,“最佳盐度窗口”;当这些参数低于这些阈值时,囊肿萌发减少。还必须存在从沉积物到表面的囊肿运输机制;在Barqueiro的ria中发现这种机制是自然的(潮流),或者在Ares和Coruña的rias中是人为的(疏浚)。在1993-2008年期间,夏季的表面温度通常有利于囊肿萌发(85%至100%);但是,低于10m深度的水温很少达到17°C的阈值(2%至18%)。在这16年期间,疏浚活动可以解释记录的开花事件的71%(Coruña)和44%(Ares)。当盛开在初夏时,有利条件没有导致新的赤潮,可能是由于囊肿发芽所需的滞后期。此外,由于特定年份的夏季上升流脉冲,当水柱中仍有高密度的硅藻(>1,000,000个细胞·L-1)时,不会出现水华。在这项研究中发现的温度-沉积物干扰模式为预防这种鞭毛藻赤潮导致的最终风险提供了有用的工具。
    Despite the fact that the first red tide reported on the coasts of the Iberian Peninsula was due to Lingulodinium polyedra, knowledge about their frequency and, particularly, about the environmental conditions contributing to bloom initiation is still scarce. For this reason, L. polyedra bloom episodes were observed and studied in three Galician rias during the summer season based on the 1993-2008 record database period; additionally, samples were collected in summer 2008. Proliferations of L. polyedra occurred in the rias of Ares and Barqueiro in June and August, respectively, while in the Ria of Coruña, they persisted from the end of June to early September. Red tides developed when the surface temperature reached 17 °C, with \"seasonal thermal window\" conditions, and when salinities were ≥30, i.e., an \"optimal salinity window\"; when these parameters were lower than these thresholds, cyst germination decreased. A cyst transport mechanism from sediments to the surface must also exist; this mechanism was found to be natural (tidal currents) in the ria of Barqueiro or anthropogenic (dredging) in the rias of Ares and Coruña. Surface temperatures during summer were usually favorable for cyst germination (85 to 100%) during the 1993-2008 period; however, water temperatures below 10 m depth only rarely reached the 17 °C threshold (2 to 18%). During this 16-year period, dredging activities could explain 71% (Coruña) and 44% (Ares) of the recorded bloom events. When a bloom episode developed in early summer, favorable conditions did not lead to a new red tide, probably due to the lag period required by cysts for germination. Moreover, blooms did not develop when high densities of diatoms (>1,000,000 cells·L-1) remained in the water column as a result of summer upwelling pulses occurring in specific years. The temperature-sediment disturbance pattern found in this study provides a useful tool for the prevention of eventual risks resulting from red tides of this dinoflagellate.
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  • 文章类型: Journal Article
    背景:由于免疫调节机制,囊性包虫病(CE)囊肿可能持续数十年。这里,我们描述了CE患者的囊肿和血液免疫反应。
    方法:我们招募了61名CE患者和19名对照受试者。我们接受了7名CE患者和一名需要进行肝膀胱切除术的对照受试者的组织样本。进行了周皮和周围肝脏中免疫细胞亚型和细胞因子的免疫组织化学评估以及全血的抗原B(AgB)特异性反应分析。
    结果:在CE中,周膜和周围的肝实质显示CD3+T淋巴细胞聚集,主要是CD4+。肝组织中存在B淋巴细胞聚集体。很少观察到单核细胞/粒细胞。Th2细胞因子表达很少,而IFN-γ表达存在于CE组织中。对照受试者未显示炎性浸润。与对照组相比,CE患者对AgB的IL-4特异性反应增加,这一结果在更大的队列中得到了证实(p=0.003),而两组的IFN-γ应答相似(p=0.5570)。
    结论:在CE患者中,CD4+淋巴细胞以低IL-4/IL-13表达水平和中等IFN-γ表达水平浸润周膜及周围肝组织;此外,在外周检测到IL-4寄生虫特异性应答。这些结果支持外膜参与CE免疫发病机制。
    BACKGROUND: Cystic echinococcosis (CE) cysts may persist for decades because of immune modulation mechanisms. Here, we characterize the cysts and the blood immune responses in patients with CE.
    METHODS: We enrolled 61 patients with CE and 19 control subjects. We received tissue samples from seven patients with CE and a control subject requiring liver cystectomy. The immunohistochemistry evaluation of the immune cell subtypes and cytokines in the pericysts and surrounding liver and the antigen B (AgB)-specific response analysis of whole blood were performed.
    RESULTS: In CE, the pericyst and the surrounding liver parenchyma showed aggregates of CD3+ T lymphocytes, mainly CD4+. B lymphocyte aggregates were present in the liver tissue. Monocytes/granulocytes were rarely observed. Th2 cytokine expression was scarce, whereas IFN-γ expression was present in the CE tissues. The control subject did not show an inflammatory infiltrate. The IL-4-specific response to AgB was increased in the patients with CE compared to the control, and this result was confirmed in a larger cohort (p = 0.003), whereas the IFN-γ-response was similar between the two groups (p = 0.5570).
    CONCLUSIONS: In patients with CE, CD4+ lymphocytes infiltrate the pericyst and the surrounding liver tissue with a low IL-4/IL-13 expression level and a moderate IFN-γ expression level; moreover, an IL-4 parasite-specific response is detected in the periphery. These results support adventitia involvement in CE immunopathogenesis.
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  • 背景:VanderWoude综合征(VWS)是一种罕见的先天性畸形,其特征是唇和/或腭裂患者的下唇凹陷。它通过具有可变表达的常染色体显性遗传传播。
    方法:研究组由24名连续患者(13名男性和11名女性)组成,在2009年至2022年期间在一个中心进行了VWS手术。他们患有:双侧唇腭裂-6例;单侧唇腭裂-9例;唇裂-1例;孤立性腭裂-8例。
    结果:在16例(66%)中,中线两侧出现下唇凹陷,而在8个(34%)的凹坑是单方面检测到的。根据一阶段原则,在平均年龄8.6个月(SD1.4,范围6-12)进行了初次裂隙修复。在所有患者中,在平均年龄为37个月(SD11.3,范围为14-85)的初次唇裂修复后,作为单独的程序进行了下唇窝修复。该综合征的所有主要修复-裂隙缺损和下唇窝修复-的平均数量为2.46。9名患者(37.5%)由于术后美学效果不佳,需要对下唇进行额外的二次矫正。
    结论:经常需要对残余下唇畸形进行二次矫正,这表明在获得VWS引起的唇凹修复的满意结果方面存在相当大的困难。评估材料中主要手术干预的平均数量仍然很低。
    BACKGROUND: Van der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is transmitted by an autosomal dominant inheritance with variable expressivity.
    METHODS: The study group consisted of 24 consecutive patients (13 males and 11 females) with VWS operated on at a single center between 2009 and 2022. They suffered from: bilateral cleft lip and palate - 6 patients; unilateral cleft lip and palate - 9 patients; cleft lip - 1 patient; and isolated cleft palate - 8 patients.
    RESULTS: In 16 (66%) cases pits of lower lip occurred on both side of midline, while in 8 (34%) the pits were detected unilaterally. The primary cleft repairs were performed according to one-stage principle at the mean age of 8.6 months (SD 1.4, range 6-12). In all patients lower lip pits repairs were performed after the primary cleft repairs as a separate procedure at the mean age of 37 months (SD 11.3 range 14-85). The mean number of all primary repairs of the syndrome-both cleft defect and lower lip pits repairs-was 2.46. Nine patients (37.5%) required additional secondary corrections of the lower lip due to the poor aesthetic post-operative outcome.
    CONCLUSIONS: The frequent need for secondary corrections of residual lower lip deformities indicates the considerable difficulties in obtaining a satisfactory outcome of the repairs to lip pits caused by VWS. The average number of the primary surgical interventions in evaluated material remained low.
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  • 文章类型: Journal Article
    目的:小儿低度胶质瘤的切除程度主要提高了无进展生存期。在交叉下丘脑胶质瘤(CHG)中,由于相关的神经和内分泌缺陷的相关高风险,完全切除是有限的。尽管如此,手术可能在多学科团队(MDT)方法的框架中发挥作用.我们报告了两个中心关于手术选择及其对长期结局的影响的回顾性经验。
    方法:分析了2004年至2022年间手术治疗的小儿CHG患者的病历。患者特征,手术干预,组织学,和非手术治疗一起检索结果测量,如视力,内分泌功能,和生存。
    结果:共有63名患者(33名女性,包括NF-1,n=8)。首次诊断的年龄为4.6岁(范围0.2-16.9),队列随访为108±72个月。20例患者接受了活检手术治疗,43例患者的中位年龄为6.5岁(范围为0.16-16.9)。患者接受2次肿瘤手术的中位数(范围1-5)。15例患者完成囊肿引流,27例患者进行了脑室-腹腔分流术植入。非手术治疗占69.8%。在后续行动结束时,74.6%的患者病情稳定。该队列的中位Karnofsky评分为90(范围0-100)。四名患者死亡。30.2%需要激素取代,66%的患者视力受损。
    结论:小儿CHG是一种慢性疾病,因为总体生存率高,有多个进展。手术治疗仍然是提供活检的关键治疗选择,有限的肿瘤减瘤,囊肿开窗术,以及MDT决策框架内的脑积水管理。团队经验有助于减少这个具有挑战性的队列中可能的赤字。
    OBJECTIVE: The extent of resection of pediatric low-grade glioma mostly improves progression-free survival. In chiasmatic hypothalamic glioma (CHG), complete resections are limited due to the relevantly high risk of associated neurological and endocrinological deficits. Still, surgery might have its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcomes.
    METHODS: Medical records of surgically treated pediatric CHG patients between 2004 and 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival.
    RESULTS: A total of 63 patients (33 female, NF-1, n = 8) were included. Age at first diagnosis was 4.6 years (range 0.2-16.9) and cohort follow-up was 108 ± 72 months. Twenty patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range 0.16-16.9). Patients received a median of 2 tumor surgeries (range 1-5). Cyst drainage was accomplished in 15 patients, and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow-up, 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range 0-100). Four patients died. Hormone substitution was necessary in 30.2%, and visual acuity was impaired in 66% of patients.
    CONCLUSIONS: Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, limited tumor-debulking, cyst fenestration, and hydrocephalus management in the framework of MDT decision-making. Team experience contributes to reducing possible deficits in this challenging cohort.
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  • 文章类型: Journal Article
    一些前庭神经鞘瘤(VS)表现为囊性形态。众所周知,与手术治疗中的实体VS相比,这些囊性VS具有不同的风险特征。尽管如此,目前还没有一项直接的比较研究比较SRS和SURGERY在囊性VS中的有效性.这项回顾性双中心队列研究旨在分析在显微外科(SURGERY)和立体定向放射外科(SRS)的双中心研究中,囊性VS与实体VS的治疗。囊性形态定义为在介入前MRI中存在任何大小的T2高强度和钆对比剂阴性囊肿。通过确定匹配的SURGERY处理的固体VS和SRS处理的固体VS的亚组进行匹配的亚组分析。功能状态,然后比较介入后肿瘤体积大小。从2005年到2011年,在两个研究地点都接受了N=901例原发性和孤立性VS患者的治疗。其中,6%为囊性形态。囊性VS的发生率随肿瘤大小而增加:KoosI中的1.75%,KoosII中的4.07%,KoosIII中的4.84%,KoosIV发病率最高,为15.43%。与实体VS相比,囊性VS的分流依赖性明显更高(p=0.024),与实体VS相比,囊性VS患者的Charlson合并症指数(CCI)明显更差(p<0.001)。囊性VS的GTR率为87%,因此显着降低,与固体VS中的96%相比(p=0.037)。与匹配的实体VS相比,SRS后动态体积变化(减少和增加)的发生率在囊性VS中明显更常见(p=0.042)。囊性VS中SRS的肿瘤进展发生率为25%。当比较外科治疗的囊性与实性VS中的EOR时,GTR的肿瘤复发率为4%,显著低于STR的50%(p=0.042).囊性VS中的肿瘤控制优于外科手术,当高度切除级别治疗时,与SRS相比。与实性VS相比,囊性SRS的治疗反应较差。然而,当通过手术治疗囊性VS时,GTR的比率低于整体,和坚实的VS队列。在囊性VS中,患有相关术后面神经麻痹的患者人数显着增加,而不是唯一的囊性形态。囊性VS应在专门中心进行手术治疗。
    Some vestibular schwannoma (VS) show cystic morphology. It is known that these cystic VS bear different risk profiles compared to solid VS in surgical treatment. Still, there has not been a direct comparative study comparing both SRS and SURGERY effectiveness in cystic VS. This retrospective bi-center cohort study aims to analyze the management of cystic VS compared to solid VS in a dual center study with both microsurgery (SURGERY) and stereotactic radiosurgery (SRS). Cystic morphology was defined as presence of any T2-hyperintense and Gadolinium-contrast-negative cyst of any size in the pre-interventional MRI. A matched subgroup analysis was carried out by determining a subgroup of matched SURGERY-treated solid VS and SRS-treated solid VS. Functional status, and post-interventional tumor volume size was then compared. From 2005 to 2011, N = 901 patients with primary and solitary VS were treated in both study sites. Of these, 6% showed cystic morphology. The incidence of cystic VS increased with tumor size: 1.75% in Koos I, 4.07% in Koos II, 4.84% in Koos III, and the highest incidence with 15.43% in Koos IV. Shunt-Dependency was significantly more often in cystic VS compared to solid VS (p = 0.024) and patients with cystic VS presented with significantly worse Charlson Comorbidity Index (CCI) compared to solid VS (p < 0.001). The rate of GTR was 87% in cystic VS and therefore significantly lower, compared to 96% in solid VS (p = 0.037). The incidence of dynamic volume change (decrease and increase) after SRS was significantly more common in cystic VS compared to the matched solid VS (p = 0.042). The incidence of tumor progression with SRS in cystic VS was 25%. When comparing EOR in the SURGERY-treated cystic to solid VS, the rate for tumor recurrence was significantly lower in GTR with 4% compared to STR with 50% (p = 0.042). Tumor control in cystic VS is superior in SURGERY, when treated with a high extent of resection grade, compared to SRS. Therapeutic response of SRS was worse in cystic compared to solid VS. However, when cystic VS was treated surgically, the rate of GTR is lower compared to the overall, and solid VS cohort. The significantly higher number of patients with relevant post-operative facial palsy in cystic VS is accredited to the increased tumor size not its sole cystic morphology. Cystic VS should be surgically treated in specialized centers.
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  • 文章类型: Journal Article
    目的:先天性肾脏和泌尿道异常(CAKUT),经常在子宫内发现,涵盖从正常出生后肾功能到胎儿死亡的广泛结局。当前的超声检查不允许对结果的准确评估。本研究旨在显着改善基于超声的CAKUT出生后肾脏生存率预测。
    方法:对15例CAKUT胎儿的肾脏进行组织学分析,以更好地规范发育不良和囊肿的超声解释。140例CAKUT胎儿产后2年随访的超声图像注解羊水量和肾脏数目,尺寸,使用标准化超声读出的发育不良和/或囊肿。使用逻辑回归研究了超声特征和临床数据(诊断时的性别和年龄)与出生后肾功能的关联。通过质谱表征与肾脏发育不良或囊肿相关的羊水蛋白质组。
    结果:组织学,超声皮质髓质分化差与发育不良病变相关,超声高回声性与小囊肿的存在相关.在所有超声和临床参数中,羊膜体积减少,发育不良和囊肿是预后不良的最佳预测因子(奇数比=57[95CI:11-481],20[3-225]和7[1-100],分别)。与单独的羊膜体积相比,将它们组合到算法中改善了对出生后肾功能的预测(在10倍交叉验证中,ROC曲线下面积=0.92[0.86-0.98])。发育不良与囊肿相关(Cramer\sV系数=0.44,p<0.0001),但是羊水蛋白质组分析显示它们具有不同的分子起源(细胞外基质和细胞接触与细胞死亡,分别),可能解释了他们预测表现的可加性。
    结论:可以通过对超声数据进行更标准化和组合的解释来改善CAKUT妊娠的产前临床建议。
    OBJECTIVE: Congenital anomalies of the kidney and the urinary tract (CAKUT), often discovered in utero, cover a wide spectrum of outcomes ranging from normal postnatal kidney function to fetal death. The current ultrasound workup does not allow for an accurate assessment of the outcome. The present study aimed to significantly improve the ultrasound-based prediction of postnatal kidney survival in CAKUT.
    METHODS: Histological analysis of kidneys of 15 CAKUT fetuses was performed to better standardize the ultrasound interpretation of dysplasia and cysts. Ultrasound images of 140 CAKUT fetuses with 2-year postnatal follow-up were annotated for amniotic fluid volume and kidney number, size, dysplasia and/or cysts using standardized ultrasound readout. Association of ultrasound features and clinical data (sex and age at diagnosis) with postnatal kidney function was studied using logistic regression. Amniotic fluid proteome associated to kidney dysplasia or cysts was characterized by mass spectrometry.
    RESULTS: Histologically, poor ultrasound corticomedullary differentiation was associated to dysplastic lesions and ultrasound hyperechogenicity was associated to the presence of microcysts. Of all ultrasound and clinical parameters, reduced amniotic volume, dysplasia and cysts were the best predictors of poor outcome (Odd ratio = 57 [95%CI: 11-481], 20 [3-225] and 7 [1-100], respectively). Their combination into an algorithm improved prediction of postnatal kidney function compared to amniotic volume alone (area under the ROC curve = 0.92 [0.86-0.98] in a 10-fold cross validation). Dysplasia and cysts were correlated (Cramer\'s V coefficient = 0.44, p<0.0001), but amniotic fluid proteome analysis revealed that they had distinct molecular origin (extracellular matrix and cell contacts versus cellular death, respectively), probably explaining the additivity of their predictive performances.
    CONCLUSIONS: Antenatal clinical advice for CAKUT pregnancies can be improved by a more standardized and combined interpretation of ultrasound data.
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