双侧肾母细胞瘤的诊治与预后

为总结双侧肾母细胞瘤(BWT)的诊治经验，对1960年至2000年收治的10例BWT患儿的临床资料进行回顾性分析，结果显示BWT发病年龄小，病理分型良好。同时发病的6例BWT中，3例接受一侧肾切除，另侧行肾部分切除或肿瘤切除术，3例行双侧肾部分切除或肿瘤切除术。异时发病的BWT4例，首诊时全部接受患肾切除术，诊断BWT时1例行活检术后放弃治疗，2例行肾部分切除术，1例行肿瘤切除术。提示早期诊断与术前、后化疗有助于保肾及提高生存率，对BWT术后不主张积极的放疗。保留一侧2／3肾或双侧1／2肾是保证患儿长期生存与生活质量的重要因素。     

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。     

肾母细胞瘤切除术中探查对侧肾脏是必须的吗?

目的：探讨肾母细胞瘤切除术中对侧肾脏探查的必要性。方法：1979年10月至1994年12月，共81例肾母细胞瘤患儿均于术前接受B超、CT和IVU检查，所有患儿均在本院接受手术并化和放疗。15例患儿接受了MRI成像检查。结果：76例患儿术前诊断和为单侧或双侧（仅3例）肾母细胞瘤，与手术结果完全一致，4例患儿术前怀疑为神经母细胞瘤，1例疑为畸胎瘤。3例双侧者均为小肿瘤侧半肾切除和大肿瘤侧全肾切除，单侧者均未探查对侧肾脏。术后病理证实为肾母细胞瘤。根据NWTS的分期标准，Ⅰ期34例，Ⅱ期23例，Ⅲ期15例，Ⅳ期6例，Ⅴ期3例，结果：78例单侧患儿随访6－20年，5年生存率为79．49％，患儿死于转移，复发和化疗或放疗并发症，无一例患儿发现有对侧肾母细胞瘤。结论：肾母细胞瘤切除术中无必要探查对侧肾脏。     

儿童双侧肾母细胞瘤手术后复发的再治疗CSCD

目的总结双侧肾母细胞瘤手术治疗后复发的临床特点及治疗经验,以提高双侧肾母细胞瘤的总体治疗水平。方法回顾性分析首都医科大学附属北京儿童医院泌尿外科2008年1月至2020年12月收治的10例双侧肾母细胞瘤复发患儿临床资料,包括患儿初次手术情况、复发时间及部位、后续治疗方法及随访情况等。结果10例中男7例,女3例,发病年龄10~69个月,平均27.3个月。初次手术的20侧中,18侧采取保留肾单位的肿瘤剜除术;1侧因肿瘤位于肾门处,保留肾脏困难而行瘤肾根治性切除术;1侧因肾内占位为多发小瘤灶而未行手术。术后病理均为预后良好型,未见间变型。复发时间为术后2~36个月。1例为双侧复发,同时有腹膜后淋巴结转移,未再行手术,以化疗为主,后转移至纵隔,最终死亡。一侧复发的9例患儿中,1例予单纯化疗9个月,肿瘤消失;8例予再次手术,仍采用保留肾单位的肿瘤剜除术;5例无再次复发;3例二次复发患儿中,1例术后再次出现原位复发合并肺转移,再次行瘤肾切除术及肺叶楔形切除术,无瘤生存至今,其余2例因多处转移而最终死亡。术后均予化疗,化疗药物包括长春新碱、阿霉素、环磷酰胺、依托泊苷和卡铂,方案不完全一致。术后1例进行了放疗。10例患儿随访时间19~108个月,1例失访;3例死亡;1例带瘤存活;其余5例均无瘤生存,至随访时患儿血清肌酐和尿素氮均正常。结论双侧肾母细胞瘤复发后可再行肿瘤切除术(nephron-sparing surgery,NSS),同时辅以加强化疗,可有相对较好的预后。     

一期手术矫治肾输尿管重复畸形合并异位输尿管囊肿

目的 探讨分析完全性肾输尿管重复畸形合并同侧上输尿管异位囊肿的病人，治疗时是否需要同时切除病变肾和输尿管囊肿。方法 对近8年来我院收治的10名完全性肾输尿臂重复畸形合并同侧上输尿管异位囊肿的病例予以总结。结果 发现其中6名初期治疗中仅切除患侧上半肾输尿管的儿童，平均术后1年都因为膀胱内囊肿增大而再次接受了输尿管囊肿切除术。另3例病人一期同时施行了上半肾输尿管切除术、膀胱内输尿管囊肿切除术和下半肾输尿管再植术，术后随访疗效良好。结论 我们认为对类似疾病应该在切除肾输尿管的同时一并切除异位输尿管囊肿。     

胎儿横纹肌瘤型肾母细胞瘤

目的 探讨胎儿横纹肌瘤型肾母细胞瘤（ＦＲＮ）特点和恰当的治疗方法。方法 从１９５５～１９９６年３６９例肾母细胞瘤中检出ＦＲＮ１０例。回顾性分析其临床表现、病理组织学特点和预后。结果 １０例ＦＲＮ均因腹部肿大或腹部肿物入院。经术前放、化疗肿瘤不缩小。６例单侧病变做瘤肾切除,５例获随访均长期存活。４例双侧病变中１例做双侧单纯肿瘤切除,３例做一侧瘤肾切除,对侧肾部分切除或单纯肿瘤切除,随访存活２年。结论     

儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的诊治分析

目的总结儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的临床、病理特点,探讨合理的治疗方法。方法回顾性分析本院收治的7例儿童囊性肾瘤、6例囊性部分分化型肾母细胞瘤患儿的临床资料,包括年龄、临床表现、影像学及病理检查结果、治疗和预后。结果13例患儿中,男8例,女5例,年龄4个月至4岁,平均1岁7个月。左侧6例,右侧5例,双侧2例。腹部包块9例,B超偶然发现4例,术前均行B超和增强CT检查。5例7侧行肿瘤剜除术,8例行瘤肾切除术。术后随访6个月至7年,未见肿瘤复发,保留肾脏的5例中,7侧残肾功能良好。结论囊性。肾瘤和囊性部分分化型肾母细胞瘤患儿术前无法鉴别,手术完整切除是主要的治疗方法,肿瘤位于肾脏一极或双侧者可行保留肾脏的肿瘤剜除术。囊性肾瘤为良性病变,术后无需化疗,囊性部分分化型。肾母细胞瘤为低度恶性或潜在恶性,Ⅰ期者可单纯手术治疗,Ⅱ期以上需行手术＋化疗。     

儿童囊性肾瘤七例北大核心CSCD

目的探讨儿童囊性肾瘤的临床、病理特点,提高对该病的认识。方法回顾性分析2006年1月至2012年6月我院收治的儿童囊性肾瘤7例的临床资料,包括患儿年龄、临床表现、影像检查、病理,治疗和预后。结果3例4侧行肿瘤剜除术,4例行瘤肾切除术。术后随访6个月～6年,未见肿瘤复发,保留肾脏的3例4侧残肾功能良好。结论囊性肾瘤是临床罕见的良性肿瘤,与囊性部分分化型肾母细胞瘤术前无法鉴别,手术切除是主要的治疗方法,术后无需化疗。     

儿童双侧肾母细胞瘤手术治疗的探讨

目的 总结探讨儿童双侧肾母细胞瘤综合治疗的疗效及随访结果。方法 回顾性分析我院白1998年8月至2010年8月手术治疗的双侧肾母细胞瘤患儿临床资料。结果 7例双侧肾母细胞瘤中,男性患儿3例,女性患儿4例,发病年龄最大21个月,最小6个月,平均年龄( 12.71±4.89)个月。临床表现腹部肿块7例(7/7),血尿1例(1/6),贫血2例(2/6),4(4/7)例活检明确病理经术前化疗后手术,3例先手术后化疗,1例化疗2个疗程疾病进展,家属放弃治疗后死亡,5例结束整个疗程随访中,4例带瘤生存,1例随访7个月后残余肿瘤病灶消失,随访时间最长10年,最短10个月,1例术后化疗中,2年存活率85.7％。结论 儿童双侧肾母细胞瘤通过手术、化疗及放疗综合治疗,可以带瘤生存。     

儿童双侧肾母细胞瘤诊治分析

目的探讨儿童双侧肾母细胞瘤(bilateral wilms tumor, BWT)的临床特点、治疗效果以及预后, 加深临床认知。方法收集北京儿童医院在2008年1月至2019年12月收治的43例BWT患儿的临床资料, 包括年龄、临床表现、影像学检查、术前化疗、手术方式、病理诊断和预后。43例共86侧肾脏;男24例, 女19例;就诊中位年龄为17个月, 范围为3～69个月;术前化疗30例(60侧)作为术前化疗组, 未化疗9例(18侧)作为未化疗组, 余4例(8侧)资料不详不列入任何组别;6例化疗前行穿刺活检。30例(60侧)术前化疗患儿中术前化疗反应评估资料完整26例(52侧), 缺失4例(8侧)。术前化疗时间范围为4～12周, 术前化疗方案以VA方案(长春新碱+更生霉素)为主, 共19例(38侧);VAD方案(长春新碱+更生霉素+阿霉素), 9例(18侧);仅运用长春新碱化疗, 2例(4侧)。本研究病例随访数据采用Kaplan-Meier法绘制生存曲线, SPSS 24.0软件计算4年无事件生存率和总体生存率。结果行双侧保留肾单位手术(nephron sparing surgery, ...     

16例双侧肾母细胞瘤的危险因素分析

目的 分析双侧肾母细胞瘤的危险因素。方法 用Kaplan-Meier生存分析方法对我院从1960～2002年收治的16例双侧肾母细胞瘤进行生存时间研究。结果 是否伴发畸形、是否术前化疗、病理分型为影响双侧肾母细胞瘤生存的危险因素；本组病例五年生存率为37．05％。结论 伴发畸形的双侧肾母细胞瘤预后较差。术前常规化疗是提高双侧肾母细胞瘤长期生存率的重要因素。根据组织学类型和临床分期，严格按照NWTS-5的标准，以使治疗个体化，是提高双侧肾母细胞生存率的重要因素。     

Extrarenal Wilms tumour.

INTRODUCTION: Nephroblastoma is one of the most common solid tumours in children. It also is the most frequent tumour found in the kidneys. In 5 % of cases it affects both kidneys at the same time. About 70 - 80 new cases of Wilms tumour are registered in Poland annually, usually in patients aged from 1 to 7 years. Extrarenal Wilms tumours are extremely rare. Due to its rarity, series with more cases are based upon material collected from many clinical centers. AIM: We would like to present a case of a boy in whom we diagnosed nephroblastoma in the retroperitoneal space 14 years after he had completed a complex therapy for bilateral Wilms tumour. CONCLUSION: The development of an extrarenal tumour 14 years after complex treatment for bilateral nephroblastoma is related to the survival of metanephros located outside the kidney.     

Nephron-sparing procedures in 11 patients with Wilms' tumor

PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed. The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation. MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination. In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium. All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients. RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range. In six patients primary PN was performed successfully. In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN. In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected. This patient evolved a local relapse 19 months after PN and had to be nephrectomised thereafter. In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis. One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively. The other two stage V tumor patients have creatinine clearance levels within the normal range. CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed. PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed. Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy. PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.     

动脉化疗栓塞术对小儿肾母细胞瘤细胞凋亡和增殖的影响

目的探讨动脉化疗栓塞术(TACE)对肾母细胞瘤(WT)细胞凋亡指数(AI)和细胞增殖指数(PI)的影响,并评价其在临床治疗中的地位。方法将24例肾母细胞瘤患儿按临床分期、病理分型进行配对设计研究,设动脉化疗栓塞组12例,全身化疗组12例,采用TUNEL法和免疫组织化学超敏二步法测定标本的细胞凋亡指数和细胞增殖指数。结果动脉化疗栓塞组AI高于全身化疗组(P<0.05),PI低于全身化疗组(P<0.05),AI与PI成负相关(P<0.05),动脉化疗栓塞组术后瘤体缩小,坏死程度高于全身化疗组(P<0.05),而骨髓抑制少(P<0.05)。结论与全身化疗组相对比,术前采用动脉化疗栓塞法能更有效地诱导肿瘤细胞凋亡,抑制肿瘤细胞增殖,促使肿瘤缩小、坏死,有利于手术根治,取得更好的疗效,提高生存率。     

Diagnostic and therapeutic strategy of bilateral nephroblastoma (apropos of 4 cases)

Four cases of bilateral nephroblastoma out of a total number of 99 cases have been observed during a period of 18 years at the INSE of Tunis (4%). The frequency of associated anomalies, the familial incidence, the young age of the patients are underlined. The main therapeutic modalities are discussed. Based on the most recent studies published in the literature, the prognosis of bilateral nephroblastoma has been improved in recent years with a possibility of survival at 2 years exceeding two thirds of the cases.     

Nephroblastoma in children aged less than 6 months at diagnosis

We present the results of treatment of kidney tumours in newborns and infants aged less than 6 months, in the years 1993-2000, from the Nephroblastoma Committee of the Polish Paediatric Group of Solid Tumours (PPGGL). We have analysed the diagnostic and treatment results in the group of 31 children aged 0 to 6 months. For 19 children registered between 1993 and 1996, event-free survival (EFS) and overall survival (AS) were assessed. Among 450 children registered between 1993 and 2000 by PPGGL and treated for kidney tumours, there were 31 (7.1%) newborns and infants aged below 6 months. The accuracy of diagnosis based on imaging studies was 97%. Only in one child the initial diagnosis of kidney tumour was not confirmed; cystic degeneration of kidney was finally established. The tumours removed during surgery were small, with average size 213 cm3, and in half of the cases the size of the tumour did not exceed 165 cm3. Primary complete excision of the tumour was performed in 21 children (67.7%). In 10 cases histopathology confirmed mesoblastic nephroma, in 19 cases nephroblastoma and in 2 cases sarcoma clarocellulare. In 10 infants (32.2%) with nephroblastoma delayed surgery preceded by chemotherapy was performed. Indications for initial preoperative chemotherapy comprised: tumour in a single kidney, tumour in a horseshoe kidney, preoperative diagnostic biopsy of the tumour and large tumour in neonates older than 3 months. In almost 70% of the children the stage of advancement was low (stage I and IIN-). Histopathology of excised tumours confirmed in 42% of cases low risk, and in 51.6% intermediate risk. Intraoperative complications occurred in 5 infants (16%). The tolerance of reduced chemotherapy by the infants was good. AS was 100%. ESF for the 19 children registered for nephroblastoma between 1993 and 1996 for all stages of advancement and types of histology was 94.75%. Conclusions: 1) Mesoblastic nephroma and low risk nephroblastoma are the most common tumours in children within the first three years of life. 2) The results of treatment of nephroblastoma in the youngest children (below 6 months of age) are the most favourable and represent world standards.3) Surgical complications in children operated primarily for nephroblastoma indicate the need of performing such operations in academic centres, specialised in newborn surgery. 4) In infants with extensive kidney tumours older than 3 months, primarily considered as inoperative, individual induction chemotherapy should be taken into account.     

A Clinical Perspective of Bilateral Renal Tumors - Data from Three Consecutive German Cooperative Trials

PurposeThe therapeutic approach for bilateral renal masses is different to unilateral renal masses. This is due to biological facts such as a remarkably higher number with nephroblastomatosis, but also to the fact that clinicians must keep drug doses as low as possible to prevent side effects.Material and MethodsWe analysed the prospectively collected data of 138 patients with bilateral renal masses registered in the consecutive German national trials SIOP9/GPO, SIOP93-01/GPOH and SIOP2001/GPOH from January 1989 to May 2005.ResultsThe median follow up was 6.2 years. All but one patient already had bilateral masses at diagnosis. Median age at diagnosis was 1.9 years with a female/male distribution of 1.5/1. 18 (13%) patients had distant metastases at diagnosis. 25% of all registered patients had a concomitant syndrome. Aiming to reduce the tumor mass as much as possible, 132 patients received preoperative chemotherapy according to the trial protocols for stage V, 6 patients underwent initial surgery. Preoperative treatment duration in the nephroblastoma group ranged from 1 to 12 weeks, leading to an average volume reduction of 44%. 11 patients with bilateral nephroblastomatosis suffered from progression, 3 eventually died from nephroblastoma. 2y/5y EFS and OS for the nephroblastoma group were 82.7/71.5% and 89/85.7%, respectively. Metastases at diagnosis, local stage III and anaplasia in histology had a negative impact on outcome.ConclusionsFive treatment-associated deaths including one acute renal failure underline the importance of a cautious approach in the complex treatment of bilateral renal tumours, including nephron-sparing surgery. This aim often can be facilitated by multimodal preoperative treatment (e.g. 1, 2 or 3 four-weeks courses of actinomycin, vincristine and doxorubicin) tailored to the individual tumor status defined by imaging after each cycle.     

Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis?

BACKGROUND: The current standard in the treatment of nephroblastoma is preoperative chemotherapy based on radiological appearance. After subsequent surgical removal few tumours proved histologically to be neuroblastoma. We asked whether initial chemotherapy according to nephroblastoma trials would change the prognosis for those neuroblastoma patients. RESULTS: Out of 1603 patients registered in the German neuroblastoma trials, 29 patients (1.8 %) have preoperatively been treated according nephroblastoma protocols. Advanced stages (11 stage 3, 12 stage 4) were dominant. Diagnostic work up of those patients revealed elevation of catecholamine metabolites in only 39 % (compared to 80 % of the control patients) and mIBG uptake in only 71 % (compared to 89 % of the control patients). Elevation of NSE was observed in 92 % of patients (control group 72 %). Patients with preoperative nephroblastoma treatment were older than the patients of the control group. Risk factors like MYCN amplification or elevation of LDH were more often detected. The outcome of the patients with preoperative chemotherapy according nephroblastoma trials was worse than that of the control group, but risk group adapted survival analysis revealed no disadvantage. CONCLUSION: The prognosis of children with neuroblastoma tumours, which have been radiologically classified as nephroblastoma, is inferior compared to the prognosis of patients without preoperative nephroblastoma therapy. The difference appears to be associated rather with more unfavourable biology than with the element "preoperative chemotherapy".     

Management of pediatric renal tumor: Past and future trials of the Japan Wilms Tumor Study Group

The Japan Wilms Tumor Study group (JWiTS) was founded in 1996 to improve outcomes for children with renal tumor in Japan, and a nationwide multicenter cooperative study was initiated thereafter. JWiTS‐1 (1996–2005) was analyzed, and JWiTS‐2 (2005–2014) is now under analysis; the following problems have been identified and used to decide future study protocol: (i) there has been a decline in survival rate for patients with rhabdoid tumor of the kidney (RTK) and new treatment strategies are required; (ii) the survival rate for bilateral Wilms tumors (BWT) has improved, but results for renal preservation are unsatisfactory; (iii) the prognosis of stage IV favorable nephroblastoma is very good, suggesting that the current protocols provide overtreatment, particularly for patients with lung metastasis; and (iv) no effective biological risk factors exist for predicting the outcome of Wilms tumor, and a study of the genetic changes of these tumors is necessary to determine biological markers for use in risk classification. To solve these issues, the development of a new risk classification of pediatric renal tumors is required. In addition, different study protocols should be developed according to the risk‐based classification of the patients. Further, a new study protocol for BWT began in 2015, and new study protocols are being prepared for RTK, and for Wilms tumor with lung metastasis. In addition, an analysis of biological markers with regard to risk classification is to be performed. Furthermore, to create new protocols for patients with rare renal tumors, international collaboration with Children's Oncology Group and International Society of Pediatric Oncology is necessary.