小儿肾母细胞瘤的治疗与预后

肾母细胞瘤是小儿最常见的胚胎性恶性肿瘤，是小儿肾脏特有的肿瘤。１９８２年１１月～１９９１年１２月共收治小儿肾母细胞瘤２１例，随访１～５年，５年存活者７例，占本组病例的３３％；２年存活７例，存活率为３３％。重点讨论了肿瘤发病年龄与预后的关系、临床病理分期与预后的关系、应用化疗与预后的关系。     

儿童肾母细胞瘤的保肾治疗

 【目的】探索儿童肾母细胞瘤（WT）保肾治疗方案和提高WT患儿长期生存率、改善生存质量的诊疗方案。【方法】根据诊治方案不同，将我院261例WT患儿分为A、B两组，通过比较A、B两组的疗效．分析外科、病理、影像、化疗、放疗等学科在提高WT疗效．改善长期生存质量中的作用以及多学科协作综合治疗对保肾治疗的作用。【结果】A组WT诊治以影像学诊断＋手术治疗为主，B组WT诊治强调明确的病理分型与临床分期以及个体化的多学科综合治疗方案。A、B两组WT患儿在2年／5年生存率、保肾治疗率、手术并发症及长期生存质量等方面均存在显著差异。对B组WT患儿中的Ⅰ期各型、Ⅱ期FH型肿瘤位于患肾一极和Ⅴ期WT患儿进行保肾治疗后，肾切除率从A组的19．1％（Ⅰ．Ⅳ期）和50％（Ⅴ期）分别下降为10．5％和30％。显著增加了保留部分患肾的机会与残肾量，改善了患儿生存质量。保肾治疗不影响患儿的肿瘤复发率。【结论】术前准确的分期分型诊断与合适的化疗是保肾治疗的关键，多学科协作诊治能显著改善WT患者的疗效：对符合保肾治疗指征的儿童WT患者进行保肾治疗，能改善WT患儿生存质量。     

肾母细胞瘤15例临床病理分析

目的 探讨肾母细胞瘤的临床病理特点及与预后的关系。方法 对15例进行临床病理分析。结果 本病占同期肾脏恶性肿瘤的41．9％，儿童肾脏恶性肿瘤的90％。发病年龄在5岁以前的为77．8％。1～3岁最多见占60％，成人少见为13．3％。结论 上皮型预后好，肉瘤型恶性度高。明确分型对估计预后有意义。     

NOV对大鼠神经干细胞增殖和分化的影响

目的：探讨NOV基因和蛋白对大鼠神经干细胞（NSCs）增殖和分化的影响。方法：NOV基因重组质粒转染COS－7细胞和NSCs，收集COS－7细胞和NOV基因修饰COS－7细胞的条件培养液（简称COS－CM和NOV－CM），作用于培养的NSCs，^3H-TdR掺入检测NSCs的增殖，免疫细胞化学和流式细胞仪检测NSCs的分化。结果：①NOV－CM能明显促进NSCs对^3H-TdR的摄入，说明NOV－CM能明显促进细胞的增殖，另外NOV－CM的促细胞增殖作用具有一定的量效关系；②免疫细胞化学和流式细胞仪结果显示，NOV－CM促进NSCs向神经元方向分化；③NOV基因修饰NSCs分化时，神经元的比例增高。结论：NOV可能具有促进NSCs增殖和分化的作用。     

Inguinal nephroblastoma

Summary A case of an extrarenal nephroblastoma is reported which was located in the inguinal region. In the surrounding fat tissue normal ectopic glomerular and tubular structures and two more nephroblastomas were found. Therefore and because of negative clinical investigations in regarding of a renal malignant tumour the inguinal mass was thought to be the primary tumour and not a metastasis. This conclusion is important for the diagnosis and therapy.Chirurgische Abteilung des Städtischen Krankenhaus Kemperhof, D-5400 Koblenz, Federal Republic of Germany     

Adult Wilms'' tumor mimicking hemorrhagic renal cyst

A 36-year-old man presented with macroscopic hematuria associated with right flank pain. Examination of the patient revealed a cystic mass in the right kidney. Because the mass had increased in size, enucleation of the mass was performed. Histopathological findings revealed nephroblastoma, therefore, radical nephrectomy was performed. We believe the pathogenesis of the cystic formation to be a process in which a tumor that had developed in the pericalyceal region spontaneously ruptured, exuding urine into the perinephric space, forming a cystic mass. The patient is alive with no evidence of disease 24 months after the operation.     

神经母细胞瘤、多形性胶质母细胞瘤、尤文肉瘤及肾母细胞瘤中全基因组关联分析计量和热点研究

[目的]分析神经母细胞瘤、多形性胶质母细胞瘤、尤文肉瘤及肾母细胞瘤全基因组关联研究（GWAS）英文文献的数据构成特征。[方法]对国际最新GWAS研究数据库之一——美国国立人类基因组研究所（National Human Genome Research Institute,NHGRI）数据库2008年11月25日至2013年8月26日收录的神经母细胞瘤、多形性胶质母细胞瘤、尤文肉瘤和肾母细胞瘤相关文献基本特征、第一阶段样本量、二阶段样本量、染色体区域相关单核苷酸多肽性（SNP）、对照组相关风险等位基因频率、P值文献中最强SNP、优势比（odds ratio,OR）或β相关系数和实验平台及检测SNP数量8项数据进行分析。[结果]共检索7篇4种肿瘤GWAS研究文献（神经母细胞瘤3篇,多形性胶质母细胞瘤1篇,尤文肉瘤1篇和肾母细胞瘤1篇）,涉及27个SNP（神经母细胞瘤10个,多形性胶质母细胞瘤1个,尤文肉瘤3个,肾母细胞瘤13个）,以在Nat Genet发表的文献最多。文献第一阶段病例组样本量从315例增加到2101例,对照组从1879例增加到3851例;第二阶段病例组样本量从434例增加到1488例,对照组从199例增加到3851例。染色体区域相关SNP以2、5、6和11号居多。对照组相关风险等位基因频率以0.40-组最多,未发现稀有变异。最强SNP相关风险等位基因P值以1×10-10-为主,最小P值为2×10-26。OR或β相关系数范围为1.23-2.64。研究平台以Illumina为主,占85.71%。检测SNP数量从286 966个到599 255个。[结论]2、5、6和11号染色体区域SNP多态性可能是神经母细胞瘤、多形性胶质母细胞瘤、尤文肉瘤及肾母细胞瘤遗传易感区域,深入此易感区域研究可能为阐述肿瘤发生、治疗及预后评估提供一定线索。     

URACHAL TUMOR: An Unusual Presentation of Neuroblastoma

The possibility of an interaction between gender and fetal growth on the risk of Wilms tumor is poorly examined. Using nationwide population-based registries, the authors identified all children (= 126) diagnosed with Wilms tumor in Denmark, 1973–1993, and selected 10 matched population controls per case. Data on birth weight, gestational age, and ponderal index were used to examine the association between gender, fetal growth, and risk of Wilms tumor. It was found that the risk of Wilms tumor increased with fetal growth in girls, but decreased with fetal growth in boys, although the findings did not reach statistical significance.     

SIOP PODC: Clinical guidelines for the management of children with Wilms tumour in a low income setting

Wilms tumour is a relatively common and curable paediatric tumour. Known challenges to cure in low income countries are late presentation with advanced disease, malnutrition, failure to complete treatment and limited facilities. In this article, management recommendations are given for a low income setting where only the minimal requirements for treatment with curative intent are available (setting 1). These include general management, supportive care, social support and registration of patients. Recommendations specific for Wilms tumour care include diagnostic procedures with emphasis on the role of ultrasonography, preoperative chemotherapy with a reduced dosage for malnourished children and postoperative chemotherapy based on surgical staging. Pediatr Blood Cancer 2013; 60: 5–11. © 2012 Wiley Periodicals, Inc.     

Wilms' Tumor with Intracardlac Extension: Chemotherapy Before Surgery

The article describes two Chinese boys ages 2 and 3 years with unilateral Wilms' tumors complicated by intracaval and intracardiac extension. In contrast to the previously recommended treatment with surgery followed by chemotherapy and radiation therapy, the children were managed primarily with combination chemotherapy before definitive operation. Reduction of tumor sire on serial imaging was documented, and no viable tumor cells were found when the involved kidney and right atrium were explored. Both patients remained alive without evidence of disease more than 5 years after initial diagnosis. A literature search revealed case reports and retrospective analyses of 70 patients with Wilms's tumors and intracardiac involvement, and a tendency toward preoperative chemotherapy with or without the addition of radiation therapj was obsemed. The overall outcome of this group of patients parallels the outcome of those without intracardiac extension ty hology and stage. Wilms's tumor presenting with extension into the inferior vem cava and right atrium is thus rare and renders the affected child with additional cardiovascular complications and operative risks. As a result of the uncommon occurrence, a consensus on managemnt based on prospective study would be dfficult. The present report and the literature are supportive of the use of preoperative chemotherapy in the initial management of advanced Wilms's tumor extending into the right atrium.