中晚期肾母细胞瘤的综合治疗(附50例报告)

目的　总结肾母细胞瘤综合治疗效果。　方法　自１９７２ 年３ 月～１９９２ 年６ 月对５０ 例肾母细胞瘤患儿采用患侧肾根治性切除术、放疗（ 根据患儿年龄,总量１ ６００ ～４ ０００ｃＧｙ）、化疗（ 长春新碱、更生霉素单独或联合应用） 的综合治疗措施。　结果　５ 、１０ 年生存率为７２％ （３６／５０） 和５３％（１８／３４）;小于５ 岁与大于５ 岁年龄组,１０ 年生存率分别为６０ ％（１５／２５） 、３３ ％（３／９）,差异有显著性（Ｐ＜０ ．０５）;术后１０ 天内与３０ 天后接受放疗者５、１０ 年生存率分别为８３ ％（１０／１２）、５８ ％ （７／１２）和５３％（８／１５）、３３％ （５／１５）,差异有显著性（ Ｐ＜ ０－０５） 。　结论　肾母细胞瘤综合治疗是提高生存率的关键。年龄越小,接受放疗时间越早,疗效越好。     

肾母细胞瘤

肾母细胞瘤(nephroblastoma,简称NB)又称Wilms’瘤,是一种复杂的胚胎性肾肿瘤,占所有肾肿瘤的6％,是小儿最常见的恶性肾肿瘤。为了解NB研究近况,加深对其临床病理特征的认识,本文主要就NE的病理、临床诊断、治疗和预后方面的文献作一综述。     

成人肾母细胞瘤的临床特点

目的 探讨成人肾母细胞瘤的临床特点.方法肾母细胞瘤患者6例.男4例,女2例.年龄21～58岁,平均31岁.临床表现为腰腹痛4例,其中伴肉眼血尿1例,伴发热1例;腰腹部肿块2例.查体腹部能触及肿块者4例,有肾区叩击痛5例,下腹部压痛1例.6例尿常规检查2例红细胞(++).6例B超检查发现5.0 cm×5.0 cm～22.0 cm×25.0 cm的低回声团块,边界尚清.4例CT检查均发现密度不均、边界不清的实质性肿块,其中1例增强后肿物不均匀强化,中心有出血坏死;CT值11～40 HU,平均22.5 HU,增强后CT值35～78 HU,平均63.5 HU.2例MRI检查,提示实性占位性病变.3例IVU检查提示肾影增大,肾盏受压变细,肾盂积水1例.术前诊断肾肿瘤5例、盆腔包块1例.6例均手术治疗.根治性肾切除术5例,卵巢切除术1例,均同时行腹膜后淋巴结清扫术.术中见肿瘤侵犯胰尾1例,侵犯侧腹壁1例,侵犯下腔静脉1例;2例肾门淋巴结＞1.5 cm×1.5 cm.剖开肿物见切面灰黄或灰白色,4例包膜完整,3例肿瘤中心出血坏死.术后接受化疗加放疗4例,单纯化疗2例.结果 按美国国家肾母细胞瘤研究组(NWTS)分期:Ⅰ期1例、Ⅱ期2例、Ⅲ期2例、Ⅳ期1例.6例均顺利手术切除肿瘤.肿瘤直径4.5 cm～25.0 cm,平均11.8 cm.术后病理确诊,其中组织学分型显示分化良好型5例,分化不良型1例.有淋巴结转移者2例,局部浸润者3例,肺转移者1例(同时存在局部浸润).术后随访1～7年,平均3.5年.5例无瘤生存,1例术后2年死于肺转移. 结论 成人肾母细胞瘤患者就诊时大多已有肿瘤转移或浸润,发生血行转移者也明显多于其他肾肿瘤,早期诊断、根治性手术及术后放化疗可提高生存率.     

成人肾母细胞瘤的临床现状

肾母细胞瘤(nephroblastoma)，又称作Wilhms瘤、肾胚胎瘤，是婴幼儿常见的恶性肿瘤，而成人发病少见。成人肾母细胞瘤诊断困难，疗效差，生存率低，3年生存率仅有24％^[1]。原因可能是由于发病零散，症状不典型，以至研究不深入，许多病例发现时已属晚期；其次可能由于发病少，没有形成明确的诊疗方案。本文对成人肾母细胞瘤的诊断和治疗作进一步的探讨。     

成人肾母细胞瘤的诊断与治疗

目的：探讨成人肾母细胞瘤的诊治方法。方法：回顾性分析7例成人肾母细胞瘤的临床资料。结果：7例均行手术治疗，6例术后辅以放疗及化疗，随访1～7年，4例仍生存。结论：成人肾母细胞瘤术前与其他肾脏恶性肿瘤难以鉴别。治疗上应尽可能手术切除肿瘤，术后根据病理分期辅以放疗和化疗。     

成人肾母细胞瘤的诊断和治疗

肾母细胞瘤是起源于未分化中胚层组织的恶性肿瘤,又称Wilm瘤、胚胎性腺肌肉瘤或癌肉瘤,是婴幼儿最多见的恶性实体瘤之一,成人罕见。我院自1970-2000年共收治成人肾母细胞瘤患者11例,现报告如下。     

肾母细胞瘤合并肾母细胞瘤病6例临床分析

目的 探讨肾母细胞瘤(WT)合并肾母细胞瘤病(Nbm)的诊治方法及预后.方法 回顾分析2006年4月至2010年7月收治的6例WT合并Nbm患儿的临床资料.男4例,女2例;发病年龄5～14个月,平均9个月.WT位于左侧4例,右侧2例; Nbm位于WT同侧3例,对侧2例,双侧1例.WT均为单发,最大径分别为3、4、8、10、11、12 cm,Nbm单发3例,多发3例.行瘤肾切除术2例,保留肾脏的肿瘤切除+对侧肾上下极结节活检术1例,瘤肾切除+对侧保留肾脏的肿瘤切除术2例,保留肾脏的肿瘤及同侧结节切除术1例.术后以长春新碱、更生霉素或阿霉素化疗.结果 1例术后化疗15个月,33个月后肿瘤复发,手术切除后5个月再次复发,继续化疗11个月后死于肝脏、肺部转移.4例术后化疗6个月,其中3例已停化疗分别9、12、21个月;1例拟化疗15个月,目前已化疗8个月.此5例目前未见肿瘤复发,仍在密切随访中.结论 Nbm恶变率高,合并Nbm的WT复发率增高,化疗可降低Nbm恶变率,恶变或化疗无效的Nbm应行保留肾脏的肿瘤切除术.

Abstract：

Objective To investigate the diagnosis,treatment and prognosis of nephroblastomatosis (Nbm) combined with Wilms'tumor (WT). Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children's Hospital from 2006 to 2010 were reviewed retrospectively.The patients'ages ranged from five to 14 months.Two of the patients were female and four were male.The WTs were left-sided in four cases and right-sided in two cases.The Nbms were ipsilateral with WT in three cases,contralateral in two cases and bilateral in one case. The Nbms were single In three cases and multiple in three cases.WTs were all single and the maximum diameter was 3,4,8,10,11,and 12 cm respectively.Two paitents underwent nephrectomy.Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases,Nephrectomy and contralateral nephron sparing surgery was conducted in an additional two cases.Adjuvant chemotherapy included vincristine,actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy.Four patients underwent 6 months of chemotherapy,and it was 9,12,and 21 months respectively after stop of chemotherapy.Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT.Chemotherapy may reduce the rate of Nbm malignancy.If Nbm is malignant or chemotherapy is invalid,nephron sparing surgery is recommended.     

术前化疗在肾母细胞瘤的应用及研究进展

术前化疗应用于肾母细胞的治疗后，存活率进一步提高到90％，术前化疗的显著临床价值，逐渐受到学者们的日益重视，并得以推广应用。本文就肾母细胞瘤术前化疗的应用及研究进展作一综述。     

Bilateral Wilms' tumor with anaplasia: lessons from the National Wilms' Tumor Study

Purpose

The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy.

Methods

One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined.

Results

Anaplasia was identified in 0 of 7 tumors by core needle biopsy, 3 of 9 tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic tumors in both kidneys.

Conclusions

Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' tumor and anaplasia.     

单纯后路全椎弓根螺钉技术矫正Wilms瘤综合治疗后并发脊柱畸形

目的探讨单纯后路全椎弓根螺钉技术矫正Wilms瘤综合治疗后并发脊柱畸形的安全性及其疗效。方法回顾分析2010年8月~2012年3月单纯后路全椎弓根螺钉技术矫正Wilms瘤综合治疗后并发脊柱畸形患者3例。分析术后畸形矫正率、并发症、融合情况及患者腰痛视觉模拟量表(visual analog scale,VAS)评分。结果术后脊柱侧凸、胸腰段后凸均显著改善。腰痛VAS评分也显著提高。均随访>2年,3例患者均无畸形明显进展,无假关节形成、感染等并发症,无疼痛症状复发。结论对于Wilms瘤综合治疗后并发脊柱畸形的患者,单纯后路全椎弓根螺钉矫形技术可取得良好的手术效果。     

Cavectomy for the treatment of Wilms tumor with vascular extension

PURPOSE: Vascular extension to the vena cava occurs in 4% of Wilms tumor cases and can reach the right atrium in up to 1%. When this happens the thrombus is usually not adherent to the vessel wall, and there is blood flow around it. Preoperative chemotherapy can cause thrombus regression and even resolution. If the thrombus persists after chemotherapy, surgery will be a challenge. On the other hand, if the thrombus invades the vessel wall, its removal may not be feasible. In this situation cavectomy is a good surgical strategy because it provides complete resection. The prerequisite for cavectomy is the absence of blood flow in the vena cava on preoperative Doppler ultrasonography. We report 3 cases of Wilms tumor with vena caval invasion in which cavectomy was performed, and discuss the principles, indications and operative technique. MATERIALS AND METHODS: A total of 171 patients with Wilms tumor were treated at our institution between 1984 and 2004. Of these patients 6 with intravascular extension of thrombus within the right atrium were treated with extracorporeal circulation, cardiac arrest and profound hypothermia, and 3 were treated with cavectomy. RESULTS: There were no instances of surgical complications or postoperative renal failure in our patients who underwent cavectomy. All remain well and free of disease. CONCLUSIONS: Cavectomy is a safe procedure for treating pediatric patients with Wilms tumor when there is extension and invasion of the vena cava wall without blood flow.     

大肠类癌的诊断与治疗

目的探讨结直肠类癌的预后因素及外科治疗方法的选择.方法回顾性分析1990～1999年经我院治疗的46例结直肠类癌的临床资料.结果大肠类癌男女之比为1.4∶1.直肠类癌发病率高(83%,38/46),肿瘤体积小(直径<2*!cm者占82%,31/38),预后较好.肿瘤直径大于2*!cm的直肠类癌转移率为71%(5/7),平均生存时间25个月.结肠类癌发现晚,转移发生早,转移率为83%(5/6),大于2*!cm结肠类癌平均生存时间18个月.结论结直肠类癌的大小,有无浸润及远处转移,是判断结直肠类癌良恶性、决定手术方式和预后的主要依据.     

重症急性胰腺炎治疗方案的探索

目的 探讨重症急性胰腺炎的治疗原则和方法。方法 对不同阶段治疗急性胰腺炎的原则和方法进行了回顾性对比分析,将１８年的治疗大致分为３个阶段。第Ⅰ阶段（１９８０年～１９９０年）以早期手术治疗为主,中西医结合治疗为辅;第Ⅱ阶段（１９９１年～１９９３年）在中西医结合治疗的基础上,强调早期并发症的中转手术治疗;第Ⅲ阶段（１９９４年～１９９７年）以中西医结合的治疗为主,手术主要针对病程后期的局部感染并发症的治     

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目的 探讨多种血清肿瘤 民物联合检测在胰腺癌诊治中的应用价值。方法 采用荧光免疫分析法和放射免疫法检测胰腺癌病人的血清CA50、CA19－9、CA125和CA242的表达，重点动态观察胰腺癌诊治过程中肿瘤标志物的表达及其临床随访价值。结果 CA19－9、CA242、CA50和CA125平行联合检测可提高胰腺癌诊断的敏感性（90.2%），系列联合检测可提高其特异性（93.5%）；结合电子束断层摄影（EBCT）检查，更可增加胰腺癌诊断的敏感性（98.6%）和特异性（94.6%）。动态监测术后多种肿瘤标志物有助于肿瘤复发和转移的随访。结论 动态联合检测CA19－9、CA242、CA50和CA125结合EBCT检查，可增加胰腺癌诊断的敏感性和特异性。     

良性前列腺增生症规范化治疗方案的多中心临床研究

目的 比较不同种类药物治疗良性前列腺增生（BPH）的疗效与差异,确定不同药物对不同患者的最佳适应证。方法 采用随机平行对照、多中心临床研究方法,对2002年9月至2003年12月906例BPH患者,随机进入选择性α-受体阻滞剂特拉唑嗪、多沙唑嗪、坦索罗辛与萘哌地尔;50α-还原酶抑制剂非那雄胺与爱普列特以及植物制剂舍尼通等7种治疗药物组。每3个月随访一次,国际前列腺症状评分（IPSS）与生活质量评分（QOL）,最大尿流率（Qmax）与平均尿流率（Qave）,前列腺总体积（TPV）与前列腺移行带体积以及残余尿量为观察指标进行疗效评价。根据不同指标基线水平将患者进行分层,比较各治疗组患者主观指标IPSS和客观指标Qmax的改善情况。结果 基线指标分析显示,全组主观指标IPSS评分和客观指标Qmax水平与TPV以及移行带体积呈明显相关性（P〈0．01）。至随访6个月时各类药物均使BPH患者的主观指标IPSS与QOL评分及客观指标Qmax与残余尿量有明显改善。各种药物对主客观指标的影响程度的组间比较显示,对IPSS的改善无显著差异;5α-还原酶抑制剂类药物爱普列特与非那雄胺可以使TPV和移行带体积均明显缩小（P〈0．05）。将患者以前列腺体积〈35．5cm^3和≥35．5cm^3分为两层,在非那雄胺治疗的患者中Qmax平均增加5．7ml／s和2．2ml／s（P〈0．01）,在舍尼通、萘哌地尔及多沙唑嗪治疗组,≥35．5cm^3者症状改善更为明显（P〈0．05）。以IPSS〈20分和≥20分进行分层,各种药物的疗效均在≥20分时更为明显（P〈0．01）。结论 各种药物均可明显改善BPH患者的主、客观症状,各种药物的疗效均对基线IPSS评分较高的患者疗效更为明显。5α-还原酶抑制剂能明显减小前列腺体积,对于前列腺体积≥35．5cm^2者有更为明显的主客观疗效。