术前化疗对肾母细胞瘤瘤体的影响

目的　探讨术前化疗对肾母细胞瘤瘤体大小及瘤体耐药的影响。方法　本组 37例 ,术前影像学分期 ,Ⅰ～Ⅱ期 ,手术切除 ;Ⅲ～Ⅴ期 ,术前化疗 2～ 6周。免疫组化检测术后瘤体组织P 糖蛋白 (P glycoprotein ,P gp)、谷胱甘肽 硫转移酶 (glutathione s transferase π ,GST π)、拓扑异构酶Ⅱ(topoisomeraseⅡα ,TOPOⅡ )表达状况。结果　影像学分期 ,Ⅰ～Ⅱ期 2 0例 ,Ⅲ～Ⅴ期 17例。术前化疗组 ,瘤体缩减率 :1～ 2周为 (18.84± 3.5 4 ) %、3～ 4周为 (10 .78± 4 .2 6 ) %、5～ 6周为 (3.18±1.0 8) % ;瘤体GST π、TOPOⅡ表达阴性。瘤体在术前化疗 2周以内无P gp表达 ,4周以内有或弱阳性表达 ,4周以后P gp表达阳性。 结论　术前化疗应针对Ⅲ～Ⅴ期患儿 ,瘤体缩小主要集中于化疗 4周以内 ,超过 4周瘤体缩小不明显 ,且有引起肿瘤耐药的可能     

肾母细胞瘤术前化疗疗效与耐药性

目的：探讨肾母细胞瘤术前化疗疗效规律及耐药性的生物学特性,确认其术前化疗的合适疗程。方法：选择15例按L化疗方案接受术前化疗5周以上,资料记录完整的患儿作为术前化疗疗效研究对象,测量术前化疗不同疗程肿瘤大小;对18例未术前化疗和经不同疗程术前化疗病理档案,采用JSD－单克隆抗体对肾母细胞瘤组织多药耐药蛋白（P-glycoprotein,P-gp)进行免疫组织化学检测,了解P－gp在术前化疗不同疗程的 表达特点。结果：术前化疗2周后,肿瘤体积缩小值最明显,术前化疗4周后,其体积缩小值减缓,部分病例有逐渐增大趋势;而术前化疗疗程越长,肿瘤细胞P－gp表达越强。结论：L术前化疗方案对肾母细胞瘤的疗效肯定,疗程以2－3周后最显著。其疗效降低可能与术前化疗后瘤组织对化疗药物耐药性明显增强有关。合适疗程为3－4周。     

术前化疗在肾母细胞瘤中的应用

肾母细胞瘤是儿童时期最常见的肾脏实体恶性肿瘤,随着以手术为主的综合治疗的不断发展,肾母细胞瘤术后无瘤生存率有了显著提高,而术前化疗在其中起了举足轻重的作用。目前长春新碱、放线菌素D及表柔比星是术前化疗的基础药物。术前化疗有不同的给药途径及方法,各种方法又有不同的化疗方案,其使肿瘤缩小增加肿瘤全瘤切除率和预测肾母细胞瘤患者预后方面的作用得到了越来越广泛的认可。未来术前化疗的目标将是提高化疗有效性,降低化疗药物近期、远期毒性。     

小儿双侧肾母细胞瘤的治疗

小儿双侧肾母细胞瘤的治疗中山医科大学附属第一医院小儿外科研究室（５１００８０）刘唐彬，赖炳耀，谢家伦，刘文旭，李桂生中山医科大学病理教研组周慕珩我院１９６０年１月～１９９１年６月共收治小儿肾母细胞瘤１４８例，全部经手术及组织学诊断证实，其中双侧患者６...     

小儿双侧肾母细胞瘤治疗的探讨

目的 通过对不同时期双侧肾母细胞瘤(Bilateral WilmsTumor，BWT)治疗方法的比较，分析BWT存活率提高的原因。方法 将我院1960年～2003年收治的16例BWT以1991年为界分为两组，通过对术前、术后化疗的方法及手术方法改进的比较，分析BWT生存率提高的原因。结果 1960～1991年6例患者，1例存活，生存率16．7％，1992年～2003年10例患者5例存活，生存率50．0％。结论 通过对手术方法的改进及加强术前、术后化疗，可提高BWT生存率。     

肾母细胞瘤治疗和研究新进展

肾母细胞瘤(Wilms‘Tumour，WT)是小儿实体肿瘤中最常见的一种。近几年来，由于对晚期患儿采取综合治疗，提高了生存率。另外，随着国际间的协作，集合资料进行分析研究，定期总结疗效经验，修订治疗方案指导临床实践，不断提高疗效，也使治愈率有了很大的提高，并且也更关注患儿长期存活的生活质量，现综述如下。     

肾母细胞瘤的诊断与治疗

肾母细胞瘤 (nephroblastoma)又称威尔姆斯瘤 (Wilm′stumor,WT) ,是婴幼儿最常见的恶性实体瘤之一 ,占 15岁以下小儿泌尿生殖系肿瘤的 80 %以上 ,发病高峰为 3岁。它也是应用现代综合治疗技术最早且疗效最好的恶性实体瘤 ,由过去死亡率的 80 %以上转为目前的存活率 80 %以上[1～ 3 ] 。WT的病因为遗传异质性 ,从胚胎发育学上来说 ,持续存在的后肾胚基未能分化为肾小球及肾小管并呈不正常的增殖而发展为WT。 15 %患儿伴有先天畸形 ,如泌尿生殖系畸形、半身肥大、WAGR综合征、Denys Drash综合征、Beckwith Wied emann综合征 ,或同时患…     

中晚期肾母细胞瘤术前介入治疗的临床研究

目的 探讨介入性肾动脉栓塞化疗（transcatheter arterial chemo-embolization,TACE)作为一种术前化疗方法治疗巨大或晚期肾母细胞瘤的可行性。方法 10例巨大或晚期肾母细胞瘤术前行TACE,栓塞剂为40％碘油（或超液化碘油）0：5－0．6ml/kg、阿霉素10－15mg/m∧2（体表面积）和明胶海绵,2周后手术切除瘤肾。结果 TACE后有发热,但无骨髓抑制、肝肾功能和心电图改变,肿瘤缩小,手术时出血较少,易完整切除。治疗后有“降期”现象,有5例因肿瘤彻底坏死而术后不能确定组织类型。结论 TACE的优点是化疗药物剂量小,全身副作用少,肿瘤坏死较彻底,可将术前治疗时间缩短为2周,可作为一种术前化疗方法,单独应用或与全身用药并用于中晚期肾母细胞瘤的治疗。缺点与静脉用药术前化疗相似,有“降期”现象,且因肿瘤彻底坏死而组织分型困难。超声引导下经皮穿刺活检有助于在TACE前确定肿瘤组织类型。     

化疗对肾母细胞瘤细胞凋亡及坏死的影响

目的 了解化疗对肾母细胞瘤细胞凋亡和坏死的影响。方法 对４４例肾母细胞瘤进行随机分组,Ａ组２６例,给予ＶＡＣ术前化疗,其中８例尚加用了介入化疗,Ｂ组１８例,Ｉ期手术治疗。用ＴＵＮＥＬ技术和常规病理检查观察了全部标本的凋亡和坏死情况,并分组进行了比较。结果 术前化疗后Ａ组瘤体凋亡指数（ＡＩ）较Ｂ组明显增加;Ａ组中化疗时间超过两周的病例ＡＩ较不足两周病例也有明显上升;但死亡和获两年以上生存病例,介入和     

Results of Wilms’ tumour management in two tertiary-care hospitals in Asia

In the period 1985–1995, 87 children underwent surgery for Wilms' tumour; 16 were lost to follow-up. Of the remaining children, 27 presented with stage I disease, 11 with stage II, 12 with stage III, 14 with stage IV, and 6 with stage V. One child was not staged. The histology was favourable Wilms' tumour in 44, anaplastic in 12, unclassified in 8, clear-cell sarcoma in 4, and rhabdoid tumour in 3. Although a total nephrectomy was generally performed, partial renal surgery was performed for 6 bilateral and 4 unilateral tumours, the latter including 2 fused kidneys. Preoperative chemotherapy was employed with benefit in massive tumours, tumour in fused kidneys, bilateral tumours, and preoperatively diagnosed inferior vena caval tumour thrombi. Postoperative chemotherapy, employed in all cases, consisted of actinomycin D and vincristine with the addition of adriamycin in anaplastic and advanced-stage tumours. Ten children underwent second-line chemotherapy for disease unresponsive to the above management, but only 1 of these is currently free of disease. Postoperative tumour-bed radiotherapy, used in selected cases, prevented local recurrence in stage I and II disease. However, 20% of stage I and II patients not receiving radiotherapy developed tumour-bed recurrence. Twenty-three children have died and 5 with advanced disease and incomplete follow-up are presumed to be dead. Nine children are currently on treatment; 34 have successfully completed treatment, the disease-free survival in stages I–V being 81%, 75%, 42%, 14%, and 50%, respectively. Overall disease-free survival was 69% for Wilms' tumour of favourable histology and 50% for anaplastic tumours. The 3 patients with rhabdoid tumours and 3 of 4 with clear-cell sarcomas have died. Wilms' tumour management in the developing world is compromised by cases lost to follow-up and late presentation with massive tumours and advanced stage. Preoperative chemotherapy is advantageous in a number of cases, and postoperative radiotherapy should be deployed more frequently. Accepted: 16 December 1996     

多学科协作模式对肾母细胞瘤治疗的影响

目的　探讨肾母细胞瘤的治疗模式及提高肾母细胞瘤的治疗效果。方法　对 1998～2 0 0 1年明确诊断的 2 0例肾母细胞瘤采用多学科协作模式进行治疗 ,即以NWTS 5治疗方案为蓝本由泌尿科、血液肿瘤科、放疗科、放射科、病理科等学科共同制定统一的治疗方案 ,通过分工协作按统一模式对Wilms’瘤根据分期、病理类型及其他危险因素进行分组并按不同分组实施相应综合治疗。治疗结果与 1995年 8月～ 1998年 5月传统治疗模式下的由泌尿科单学科负责治疗 (包括手术、化疗、放疗、肾动脉栓塞、LAK细胞治疗等 )的 16例Wilms’瘤比较 ,以了解不同治疗模式对Wilms’瘤疗效的影响。结果　本组Ⅰ期 5例 ;Ⅱ期 5例 ;Ⅲ期 6例 ;Ⅳ期 3例 ;Ⅴ期 1例。病例分型FH型 14例 ;UFH型 3例 ;透明细胞肉瘤 2例和恶性肾横纹肌样肉瘤 1例。初治停药复发病例 1例 (复发率 5 % ) ,再次治疗完全缓解 ;初治失败并死亡 2例 ,病死率 10 %。目前无瘤生存 18例 (90 .0 % ) ,均已停药。对照组 :复发并死亡 6例 ,分别于术后 2个月至 8年死亡 ,复发率及病死率为 37.5 0 %。目前无瘤生存10例 (6 2 .5 0 % )。结果经统计学处理 ,二组疗效差异有显著性意义 (P <0 .0 5 )。结论　多学科协作模式下Wilms’瘤的综合治疗较果比单一泌尿科负责治疗有效、安     

The prognostic significance of macroscopic haematuria in Wilms' tumour

Of thirty-nine children over a year old with Wilms' tumour, fifteen had macroscopic haematuria at diagnosis. They had shorter survival times despite being similar to the rest in terms of treatment, age, stage of disease, histological appearances, blood pressure and the presence of abdominal pain. This suggests that haematuria complicating Wilms' tumour relates to an inferior prognosis, which, if confirmed, could be relevant to planning therapy.     

A case of bilateral Wilms' tumour of the kidneys and the retrourethral region

Bilateral Wilms' tumour of the kidneys and the retrouretheral region in a 52-day-old boy is reported. These tumours are of the same histological appearance, similar to that of a malignant rhabdoid tumour of the kidney. We suggest that these tumours have arisen from the mesonephric duct or the mesonephros and Wilms' tumour may have originated from mesonephros or an even earlier developmental stage.Abbreviations MRTK malignant rhabdoid tumour of kidney - AFP -feto protein - CEA carcinoembryonic antigen - VMA vanilmandelic acid     

Lack of association between HLA specificities and Wilm's tumour

HLA antigen distribution was determined in thirty patients with Wilms' tumour, and their frequencies compared with those of an ethnically matched control population. No statistically significant association was found between any single HLA antigen and Wilms' tumour disease.The value of prospective HLA typing studies, with special respect to genetic aspects, histopathological subgrouping and survival rate of Wilms' tumour patients is discussed.     

Improvement in outcome of children with Wilms' tumour in South Australia over the last 30 years

Objective: To evaluate the outcome of children with Wilms' tumour over the last 30 years in South Australia. To compare the outcome of children treated before and after 1982, when standard treatment protocols were introduced.
Methodology: Management approaches, survival rates and side-effects of treatment were identified from case notes. Pathology slides were reviewed to ensure all children were correctly diagnosed with Wilms' tumour.
Results: Children treated for Wilms' tumour prior to 1982 had an overall survival rate of 54%. Since 1982 there has been a significant improvement in outcome and the current survival rate is now 85%. Children treated since 1982 have also experienced fewer treatment related side-effects than children treated prior to 1982.
Conclusions: There has been substantial improvement in survival from childhood Wilms' tumour over the past 30 years in South Australia. This is likely to be due to a combination of factors including standardization of treatment, tailoring of treatment to stage and histology, improved perioperative care, enhanced radiological techniques and higher levels of collaboration between relevant specialists.     

Molecular genetics of Wilms' tumour

Wilms' tumour, or nephroblastoma, is an embryonal malignancy of the kidney with an incidence of approximately 1 in 10000 live births. It occurs in both sporadic and familial forms, but only 1% of Wilms' tumour patients have a positive family history. The molecular genetics of Wilms' tumour have been the subject of extensive research and at least three genes ( WT 1, WT 2, WT 3) have been implicated. WT 1 has been mapped to 11p13, and it has been suggested that loss or inactivation of a tumour-suppressor gene at 11p13 might be a primary event in the development of Wilms' tumour. The WT 2 gene maps to 11p15 in the region of the Beckwith-Wiedemann locus. The WT 3 locus is likely to be located to chromosome 16q. The understanding of the molecular genetics of Wilms' tumour is reviewed briefly.     

Analysis of paediatric tumour types associated with hemihyperplasia in childhood

In order to further explore the relationship between hemihyperplasia in children and the occurrence of embryonal tumours of childhood, the records at St Jude Children's Research Hospital were examined for patients who presented with a malignant tumour and hemihyperplasia. Of 27 evaluable patients, 19 had Wilms' tumour and one had massive bilateral nephroblastomatosis. The tumours were more likely to occur on the side affected by hemihyperplasia than to be found contralaterally. All but five of these patients developed the tumours before, the age of six. Twenty-two of the 27 patients developed tumours associated with allelic loss on chromosome band 11p15, suggesting that the locus associated with hemihyperplasia may be also located at chromosome band 11p15.     

Wilms' tumour with acquired von Willebrand's disease

A young infant with Wilms' tumour was found to have acquired von Willebrand's syndrome but no bleeding symptoms. Neither parent of the infant had evidence of von Willebrand's syndrome. Postoperatively, after removal of the tumour, factor VIII complex levels returned to normal. The mechanism of the acquired von Willebrand's syndrome was unclear. No evidence was found of an inhibitor of factor VIII complex or entrapment of factor VIII in the tumour.     

Perlman and Wiedemann-Beckwith syndromes: Two distinct conditions associated with Wilms' tumour

Though children with Perlman and Wiedemann-Beckwith syndromes have a number of features in common, the two conditions are probably separate entities. The distinction may not always be easy, however, partly because of the extreme rarity of Perlman syndrome, only nine cases of which have been reported so far. We report two siblings, initially diagnosed as having Wiedemann-Beckwith syndrome, in whom the correct diagnosis of Perlman syndrome was made only after an autopsy on the second child. By comparing and contrasting the features of Perlman and Wiedemann-Beckwith syndromes in this report we hope to make it easier to distinguish the two conditions.