儿童腹部神经母细胞瘤手术技术探讨CSCD

儿童神经母细胞瘤(neuroblastoma,NB)是一种发生于交感神经系统的异质性实体肿瘤,最常见于腹膜后。手术在NB多学科治疗中起着重要的作用,其主要目的是切除肿瘤病灶和尽可能保持周围组织结构的完整。关于NB的最佳手术方案目前尚未达成共识。手术入路很大程度上取决于肿块的解剖位置及对周围组织的侵犯程度。本文结合文献及作者多年临床实践,探讨儿童腹部NB的手术技术。     

Apelin/APJ在小儿神经母细胞瘤中的表达及意义

目的探讨Apelin/APJ (putative receptor protein related to the angiotensin receptor AT1)系统与小儿神经母细胞瘤(neuroblastoma,NB)发生发展的关系。方法收集10例NB患儿术后肿瘤组织及对应的癌旁肾上腺组织,采用免疫组化法检测Apelin及APJ蛋白在肿瘤及癌旁组织中的表达; Real-time PCR方法和Western blotting技术检测Apelin/APJ mRNA及蛋白的表达水平,采用ELISA方法检测NB患儿及健康儿童血浆中Apelin-36的水平。结果在收集的NB病例中,免疫组化结果显示,Apelin蛋白主要定位于神经母细胞瘤的细胞浆,呈强阳性表达,而APJ蛋白则主要在肿瘤胞核内表达,呈棕黄色颗粒,表达强阳性或中等阳性,两者均在对照组癌旁肾上腺组织中表达呈阴性或者弱阳性;与瘤旁组织比较,NB组织中Apelin、APJ mRNA和Apelin及APJ蛋白的表达水平均显著升高,且差异有统计学意义(P 0. 05)。NB患儿血浆Apelin-36的水平显著高于健康儿童对照组的水平(P 0. 05)。结论 Apelin/APJ系统在小儿神经母细胞瘤组织中高度表达,提示该系统与NB的发生发展密切相关。     

SNHG7-miR-653-5p-STAT2反馈通路在神经母细胞瘤进展中的调节作用研究

目的 研究lncRNA SNHG7在神经母细胞瘤(neuroblastoma,NB)进展中的作用,以及SNHG7-miR-653-5p-STAT2反馈通路在神经母细胞瘤进展中的调节作用.方法 从青岛大学附属医院收治的NB患儿体内获得92对NB组织及相邻非肿瘤组织.采用qRT-PCR检测SNHG7在神经母细胞瘤肿瘤组织及...     

神经母细胞瘤分子遗传学研究进展

近年来儿童恶性肿瘤发病率明显上升，已成为儿童疾病主要死亡原因之一。神经母细胞瘤（neuroblastoma，NB）是常见的儿童颅外恶性肿瘤，在儿童所有肿瘤相关死亡原因中达15％。NB临床特点为原发部位隐匿，早期无特异性症状，早期诊断困难，恶性程度高，发展迅速且易发生早期转移等。NB肿瘤起源于神经嵴组织，常发生于肾上腺髓质或脊柱旁神经节，常表现为颈部、胸部、腹部和骨盆部的肿块，原发瘤最多见的部位为腹膜后和后纵隔。     

儿童闭合性肾损伤的诊治

目的探讨儿童闭合性肾损伤的临床及诊治特点。方法回顾性分析23例儿童闭合性肾损伤的临床资料。结果23例中,16例腰痛(69.57%),21例血尿(91.30%)。非手术治疗18例,其中2例失败而中转手术;手术治疗7例,其中肾修补5例,肾切除2例,1例死于合并伤。结论血尿和腹痛是儿童闭合性肾损伤主要的临床表现;超声检查、IVU及CT能及时明确肾损伤的程度和范围;治疗原则为最大限度地保留有功能的肾组织,尽量降低肾切除率。     

SNHG7-miR-653-5p-STAT2反馈通路在神经母细胞瘤进展中的调节作用研究CSCD

目的研究lncRNA SNHG7在神经母细胞瘤(neuroblastoma,NB)进展中的作用,以及SNHG7-miR-653-5p-STAT2反馈通路在神经母细胞瘤进展中的调节作用。方法从青岛大学附属医院收治的NB患儿体内获得92对NB组织及相邻非肿瘤组织。采用qRT-PCR检测SNHG7在神经母细胞瘤肿瘤组织及细胞中的表达情况。采用Kaplan-Meier分析神经母细胞瘤患儿的总体存活率。采用比色法(MTT法)和集落形成法检测SNHG7对SK-N-SH和SH-SY5Y细胞的作用。采用Transwell侵袭及迁移试验检测SK-N-SH和SH-SY5Y细胞的侵袭和迁移能力。采用荧光素酶检测miR-653-5p和SNHG7、STAT2之间的作用。采用RIP测定及RNA下拉测定检验SNHG7和miR-653-5p在NB中的相对表达情况。采用Spearman相关分析探究SNHG7与miR-653-5p、STAT2的相关性。结果qRT-PCR显示,92对NB组织及相邻非肿瘤组织中,肿瘤组织(n=53)中SNHG7的表达量高于非肿瘤组织(n=39)。SNHG7高表达的NB患儿(n=53)总生存期随月份的增加而逐渐降低,SNHG7低表达的NB患儿(n=39)总生存期随月份的增加也逐渐降低,两组差异有统计学意义(P=0.004)。细胞功能试验:①MTT法和集落形成法检测结果显示,SNHG7的下调对SK-N-SH和SH-SY5Y细胞的存活和增殖有明显抑制作用;②Transwell试验检测结果显示,敲低SNHG7可明显抑制SK-N-SH和SH-SY5Y细胞的细胞迁移和侵袭能力(P<0.05);③荧光素酶检测显示,miR-653-5p能降低SNHG7-WT(野生型)的荧光素酶活性,且miR-653-5p与STAT2-WT(野生型)之间存在特异性的相互作用。Spearman相关分析显示:①NB组织中SNHG7与miR-653-5p表达水平呈负相关(r=-0.281,P=0.007);②STAT2的表达量与NB组织中miR-653-5p表达水平呈负相关(r=-0.295,P=0.004),STAT2的表达量与NB组织中SNHG7表达水平呈正相关(r=0.296,P=0.004)。结论SNHG7通过miR-653-5p/STAT2通路促使NB进展,这为NB提供了一个新的治疗靶点和预测预后的生物标志物。     

儿童神经母细胞瘤中ALK基因突变的研究

目的：探讨中国儿童神经母细胞瘤（NB）中间变性淋巴瘤激酶（ALK）基因的突变情况。方法：从22例石蜡包埋的NB肿瘤组织中提取基因组DNA,通过PCR-DNA直接测序技术分析儿童NB肿瘤组织中ALK突变热点区域第20~26号外显子的突变情况。结果：22 例儿童NB中,有10例（46%）在ALK第21号外显子上存在已知同义突变C3375A（Gly1125Gly）,等位基因频率为27%;1例（5%）在ALK第23号外显子上发现一个新的同义突变C3586T（Leu1196Leu）。ALK突变C3375A的发生频率在NB患儿的年龄、性别、肿瘤分化程度和是否转移方面差异无统计学意义（P＞0.05）。在其他5个ALK外显子中未发现突变。结论：采用PCR-DNA测序技术发现了ALK一个新的同义突变C3586T,并证实了儿童ALK存在已知突变C3375A,其发生率不受NB患儿临床特征的影响。     

抗神经节苷脂GD2抗体免疫治疗神经母细胞瘤研究进展

正神经母细胞瘤(neuroblastoma,NB)是儿童最常见的颅外实体肿瘤,约占小儿恶性肿瘤的8%~10%及小儿肿瘤相关死亡的15%~([1])。其起源于原始神经嵴细胞,可发生于交感神经系统的任何部位。NB具有早期转移、恶性程度高等特点~([2]),早期NB患儿可采用手术切除(1~2期)或术后给予标准剂量的化疗(3期)等治疗手段,5年总生存率(overall survival,OS)和无病生存率(event-free survival,EFS)可达(99±1)%和(90±3)%[3];而高危NB患儿(4期)尽管采用联合化疗、放疗和手术等综合治疗后,因多药耐药(multi-drug resistance,MDR)的产     

神经母细胞瘤多药耐药机制的研究

神经母细胞瘤(neuroblastoma,NB)是儿童最常见的颅外实体恶性肿瘤,临床表现多种多样,肿瘤转移及复发是其死亡的主要原因.目前NB治疗方法主要集中在手术治疗、系统化疗、放射治疗、清髓治疗联合移植、肿瘤靶向治疗等,但高危患儿的治疗效果仍不容乐观.研究表明多药耐药(multidrug resist-ance,MDR)是成功治疗NB的主要障碍,因此全面认识NB耐药机制对于提高患儿生存率具有十分重要的意义.该文从多方面就NB耐药机制进行综述.     

自体外周血造血干细胞移植在儿童神经母细胞瘤中的应用

神经母细胞瘤（Nueroblastoma,NB）是儿童最常见的外周神经来源的恶性肿瘤,约占儿童恶性肿瘤的7％～10％。根据INSS分期标准NB临床分期分为1～4期以及特殊4s期,4期恶性程度最高、预后最差。MauricioA等怛J2006年报道153例Ⅳ期NB长期生存率仅为34％。目前骨髓移植或自体外周血干细胞移植（autologous peripheral blood stem cell transplantation,APBSCT）对于治疗儿童Ⅳ期NB具有一定的临床疗效。APBSCT是经大剂量放化疗预处理,清除受体体内的肿瘤细胞及异常克隆细胞,阻断发病机制,然后移植人自体造血干细胞,使其重建正常造血免疫,而达到治疗目的的一种治疗手段。     

神经母细胞瘤并骨转移患儿临床特征及预后相关因素的单中心分析

目的 分析伴骨转移神经母细胞瘤(neuroblastoma,NB)患儿的临床特征及预后相关因素,总结临床诊疗经验,以提高伴骨转移NB患儿的生存率.方法 以2013年12月至2020年12月重庆医科大学附属儿童医院肿瘤外科收治的伴骨转移NB患儿为研究对象,收集并总结患儿临床资料及预后情况.随访时间截至2021年3月31日...     

神经母细胞瘤并骨转移患儿临床特征及预后相关因素的单中心分析CSCD

目的分析伴骨转移神经母细胞瘤(neuroblastoma,NB)患儿的临床特征及预后相关因素,总结临床诊疗经验,以提高伴骨转移NB患儿的生存率。方法以2013年12月至2020年12月重庆医科大学附属儿童医院肿瘤外科收治的伴骨转移NB患儿为研究对象,收集并总结患儿临床资料及预后情况。随访时间截至2021年3月31日。结果共收集97例NB患儿,男68例,女29例,男女比例为2.4∶1;中位年龄为49.4个月。首发症状:发热47例(48.5%),骨痛38例(39.2%),腹痛或腹胀29例(29.9%),咳嗽15例(15.5%);伴骨转移的NB患儿存在多类骨转移(62.9%),且合并骨髓转移(73.2%),整体预后差,1年生存率为93.6%,5年生存率仅20.2%。经生存分析发现,女性、伴多类骨转移、肿瘤位于腹部、首诊时LDH测定值大于660 U/L以及术中肿瘤残留是预后不佳的影响因素,其中手术切除范围是独立预后影响因素。结论伴骨转移的NB患儿临床表现多样,其预后受诸多因素影响,肿瘤复发或进展是主要的致死原因。对于此类患儿,建议术中尽可能完全切除肿瘤,以改善预后。     

Recent clinical research on the application of liquid biopsy in neuroblastoma北大核心CSCD

神经母细胞瘤（neuroblastoma,NB）是儿童最常见的颅外实体瘤,具有复发率高和生存率低的特点,因此,早期诊断、疗效评估和复发监测对NB患者至关重要。液体活检是指以血液为主的体液标本中细胞及核酸的检测,具有非侵入性并且可以克服肿瘤异质性,为实现NB的早期诊断及动态监测提供可能。该综述介绍了液体活检在NB中应用的最新临床研究进展。     

Minimally invasive surgery does not improve the outcome in a model of retroperitoneal murine neuroblastoma

Minimally invasive surgery (MIS) for cancer patients has become widely accepted in general surgery, however, it has not completely replaced the standard open operative procedures in pediatric oncology. The aim of this study was to evaluate the host relationship following MIS in a murine model of retroperitoneal neuroblastoma (NB) Immature, 5- to 7-week-old male A/J mice weighing 18–23 g were inoculated with either C1300 or TBJ NB in the left retroperitoneal space. At 4 days (early stage) or 11 days (late stage) following tumor inoculation, the animals underwent a laparotomy or pneumoperitoneum with carbon dioxide under general inhalational anesthesia. Animal survival, tumor growth, and postoperative changes in body weight were observed. In the model of subcutaneous TBJ NB, distant metastases following the laparotomy or MIS technique were also evaluated. Each surgical group had a sample size ≥12, and data were statistically analyzed by ANOVA and the chi-square test where appropriate. P<0.05 was considered to be significant. There were no significant differences in animal survival, tumor growth, or distant metastases among surgical groups in any combination of type and stage of tumor. The only salutary influence of MIS was seen in a model of early-stage NB, where the decrease in body weight on postoperative day 7 was preserved when compared to post-laparotomy weight loss. We conclude that when compared to conventional laparotomy, the MIS access technique does not influence the outcome in a model of retroperitoneal murine NB. Accepted: 11 June 1997     

Circulating tumor DNA in neuroblastoma

As a sympathetic nervous system–derived tumor, aggressive neuroblastoma (NB) is currently attracting interest from researchers seeking diagnostic and prognostic markers via less invasive procedures. The analysis of circulating tumor DNA (ctDNA) in peripheral blood can provide genetic information from multiple tumor lesions and is not dependent on a surgical procedure. The identification of genetic alterations, chromosomal variations, and hypermethylation contained within plasma DNA yields clinical value in the diagnosis, risk stratification, monitoring of treatment effects, and survival prediction for patients. With the widespread application of genome sequencing, droplet digital polymerase chain reaction, and other advanced technologies, the detection of ctDNA may guide therapeutic schedules, enhance the quality of life, and improve the prognosis for patients with NB.     

Retropharyngeal neuroblastoma in a neonate: case report and literature review

Primary cervical neuroblastoma (NB) in neonates is extremely rare. We treated a 1-day-old male neonate who presented with stridor and feeding difficulty and was subsequently diagnosed with NB of the retropharynx. The tumor was excised in toto transorally, and no metastatic lesions were confirmed. Histopathology and molecular genetic analysis showed poorly differentiated NB with no N-myc amplification, stage I NB. He has had no signs of recurrence or adverse sequelae during 18 months of follow-up. We report our experience and review the literature.     

小儿神经源性膀胱的外科治疗

目的 探讨神经源性膀胱的外科治疗方法。方法 回顾性分析我院120例神经源性膀胱的临床资料。结果 120例中获随访96例，平均随访2年，症状好转者82例。结论 对神经源性膀胱患儿的手术治疗，术前应作全面的评价，制定相应的治疗方案，才能取得满意效果。     

Case selection in minimally invasive surgical treatment of neuroblastoma

PURPOSE: The experience with minimally invasive surgery (MIS) in the treatment of neuroblastoma (NB) is anecdotal. The purpose of this study was to evaluate a retrospective cohort of NB patients who underwent MIS resection of their primary tumors. METHODS: A retrospective study of NB patients who underwent MIS resection of their primary tumors over a 3-year period was undertaken. Study outcomes included complications, completeness of resection, and event-free and overall short-term survival. RESULTS: Of a total of 21 children who underwent surgical resection for NB during the period of study, 8 (38%) underwent selected MIS resection. Six of the eight (75%) tumors were adrenal in origin and the remainder were located in the posterior mediastinum. Distribution by International Neuroblastoma Staging System (INSS) stage was: stage 1 (3), stage 2 (2), and stage 4 (3). One stage 4 tumor was N-myc amplified. All stage 4 patients experienced a >50% tumor volume cytoreduction in response to preoperative chemotherapy. All MIS resections were performed without need for blood transfusion, or conversion to open procedure, and there were no perioperative complications. All eight patients were alive and disease-free at a median 18-month follow-up. CONCLUSIONS: With appropriate preoperative case selection based on anatomic features, MIS tumor resection in patients with NB can be performed safely and effectively.