肾母细胞瘤术前化疗方案及手术时期的选择评价

目的探讨肾母细胞瘤术前化疗与手术时期的选择。方法肾母细胞瘤患儿57例，男41例，女16例；年龄2个月。11岁，平均年龄6．3岁；因腹部肿块就诊43例，肉眼血尿就诊14例；肿瘤越过中线39例；排除化疗前即存在恶病质、肿瘤侵犯大血管及远处转移病例。所有病例随机分为标准化疗组与改良化疗组，标准化疗组26例，术前按全国推荐方案进行2个疗程的化疗；改良化疗组31例，实施1个疗程化疗方案。结果标准化疗组与改良化疗组全瘤切除率分别为69．2％、93．5％。结论术前化疗可有效提高肾母细胞瘤患儿术后存活率，但全瘤切除是保障患儿长期存活的基本条件，术前化疗与手术时期的选择对肾母细胞瘤患儿无瘤存活率具有重要意义。     

金属硫蛋白在肾母细胞瘤中表达状况分析

目的　探讨金属硫蛋白 (metallothionein ,MT)在肾母细胞瘤中表达状况及术前化疗对其表达的影响。方法　本组病例 30例 ,其中术前化疗 12例 ,化疗 2周 10例 ,2周以上 2例。采用免疫组化检测技术对 30例肾母细胞瘤瘤体组织、10例瘤旁肾组织和 10例正常肾组织进行免疫组化染色 ,观察金属硫蛋白在各种组织中表达的状况。分析金属硫蛋白在肾母细胞瘤各组织类型中表达状况以及术前化疗对其表达的影响。结果　正常肾组织无MT表达 ,瘤旁肾组织镜下为正常肾组织 ,MT表达很强 ,瘤体组织较弱 ,相互比较差异有统计学意义 (P <0 .0 5 )。MT在瘤体各组织类型中的表达无明显差异 (P >0 .0 5 )。术前化疗组瘤体MT的表达高于术前化疗组 (P <0 .0 5 )。瘤体组织中MT阳性表达患儿预后优于阴性表达者。结论　肾母细胞瘤的发生对MT的表达可能有诱导机制 ,尤其对瘤体周围肾组织 ,而瘤体MT的表达可能不利于术前化疗 ,同时术前化疗可以诱导瘤体MT的表达。患儿预后与瘤体组织MT表达呈正相关。     

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。     

儿童原发性恶性非肾母细胞瘤性肾脏肿瘤诊治特点

目的 探讨儿童原发性恶性非肾母细胞瘤性肾脏肿瘤的临床诊治特点.方法 回顾性分析1993年4月至2008年1月问收治的11例儿童原发性恶性非肾母细胞瘤性肾脏肿瘤患儿的临床资料.根据临床表现及术前影像学检查并于术前行穿刺活检,治疗方法主要为术前介入和/或全身化疗、手术切除、术中热灌注化疗和术后化疗.结果 肾细胞癌6例,无瘤长期(平均32个月)生存率66.7%.中胚性肾瘤3例,均获3年以上无瘤生存.肾透明细胞肉瘤1例,术后复发死亡.肾横纹肌样瘤1例,术后化疗2个月复发,结论儿童原发性恶性非肾母细胞瘤性肾脏肿瘤发病率低,临床表现与肾母细胞瘤相似,术前诊断较为困难,其中肾细胞癌发病年龄多为年长儿,而肾透明细胞肉瘤、中胚层肾瘤和肾横纹肌样瘤则多见于小婴儿.经过多项系统性治疗,中胚层肾瘤预后较佳,肾细胞癌次之.     

肾母细胞瘤端粒酶的原位表达及临床意义

目的 检测肾母细胞瘤中端粒酶的原位表达，评价术前化疗、临床分期及组织学分型等对肾母细胞瘤端粒酶表达的影响。方法 同顾性分析复旦大学附属儿科医院外科手术切除的肾母细胞瘤标本24例。18例未接受术前化疗而直接手术，6例接受术前化疗。用原位杂交的方法检测肾母细胞瘤中端粒酶-RNA的表达。结果 24例中，端粒酶-RNA阳性表达16例(66．7％)。不同年龄、性别、组织学分型、临床分期对端粒酶-RNA表达无明显的影响。术前未化疗组胚基成分和上皮成分的表达强度相当，但明显高于间质成分的表达强度；胚基成分和上皮成分的表达强度在术前化疗组明显低于术前未化疗组，间质成分的表达强度无改变。结论端粒酶的表达在胚基细胞优势型和上皮细胞优势型肾母细胞瘤的发生过程中起了重要作用，化疗可抑制端粒酶在人肾母细胞瘤胚基成分和上皮成分中的表达。     

Ⅲ、Ⅳ期肾母细胞瘤综合治疗15年经验

目的总结近15年来对Ⅲ、Ⅳ期。肾母细胞瘤患儿的多模式综合治疗经验。方法1995年5月至2010年12月浙江大学医学院附属儿童医院共对26例单侧Ⅲ、Ⅳ期肾母细胞瘤患儿采用多模式的综合治疗。诊断标准：肾门、主动脉旁淋巴结转移;弥漫性腹腔播散或术时散落;腹膜有肿瘤种植;镜检或肉眼有肿瘤残留;局部浸润至重要脏器;肿瘤远处转移。全部病例按年限和治疗方式分为两组：①术前单纯介入治疗组（TACE组）11例,为1995年至2002年收治病例,采用术前肾动脉化疗栓塞（TACE）,1周后手术切除瘤肾,术后化疗或加放疗的综合治疗;②术前介入治疗加短期全身化疗组（T＋S组）15例,为2003年至2010年收治病例,采用术前TACE加2～3周静脉化疗,然后手术切除瘤肾,术后化疗或加放疗的综合治疗。TACE采用吡柔比星40mg／m。,长春地辛3mg／m2,超液碘油5～10mL。术前短期静脉化疗采用长春地辛3mg／（m2·周）,共2次;放线菌素D10we／（kg·d）,共5次。术后化疗和放疗按照北京儿童医院肾母细胞瘤治疗方案。TACE组与T＋s组分别有3例和9例接受术后放疗。结果两组患儿术后分期为：TACE组Ⅲ期10例,Ⅳ期1例;T＋S组Ⅲ期11例,Ⅳ期4例。两组各有弥漫问变型2例。两组肿瘤完整切除率分别为63．6％（7／11）和80．0％（12／15）,P=0．407。随访至2010年12月,两组平均随访时间分别为118（102—186）个月和43．5（1～92）个月,无瘤生存率分别为72．7％和100．0％,Kaplan-Meier生存分析显示两组差异有统计学意义（P=0．040）。结论本研究表明,术前动脉栓塞化疗加短期静脉化疗,手术切除瘤肾,术后继续化疗和放疗的多模式综合治疗是对Ⅲ、Ⅳ期肾母细胞瘤患儿的合理治疗方案。     

晚期肾母细胞瘤的术前治疗

目的　探讨晚期肾母细胞瘤术前治疗的必要性 ,并分析术前全身化疗和肾动脉化疗栓塞合并全身化疗的治疗效果。方法　 30例Ⅲ、Ⅳ期肾母细胞瘤分为 2组 :①未术前治疗组 11例 ;②术前治疗组 19例 (又分为全身化疗亚组 6例和肾动脉化疗栓塞合并全身化疗亚组 13例 )。结果　未术前治疗组手术死亡率 18.2 % (2 /11) ,肿瘤完整切除率 9.1% (1/11) ,2年无瘤生存率 9.1% (1/11)。术前治疗组手术死亡率 0 % (0 /19) ,肿瘤完整切除率 6 3.2 % (12 /19) ,2年无瘤生存率 73.7% (14 /19)。肿瘤完整切除率比较和 2年无瘤生存率比较差异均有高度显著性意义 (<0 .0 1)。术前治疗组中 ,肾动脉化疗栓塞合并全身化疗亚组完整切除率和 2年无瘤生存率均高于全身化疗亚组。结论　术前治疗可明显提高Ⅲ、Ⅳ期肾母细胞瘤的完整切除率和 2年无瘤生存率。术前肾动脉化疗栓塞与全身用药化疗并用疗效更好。     

肾母细胞瘤诊疗进展

肾母细胞瘤是儿童最常见的原发性肾脏恶性肿瘤。完整切除肿瘤和精确判断肿瘤扩散程度是决定肿瘤准确分期和后续治疗的关键。保留肾单位手术治疗双侧肾母细胞瘤安全且有效。双侧肾母细胞瘤、肿瘤并下腔静脉和心房瘤栓以及术前影像学检查容积大于1 000 mL的肿瘤,接受新辅助化疗后再手术,可降低手术并发症的风险。肾母细胞瘤新的治疗方案是在细化危险分层系统的基础上,进行个体化治疗,以保持较高治愈率,进一步降低化疗和放疗的毒性作用。     

肾母细胞瘤基因表达及治疗的研究进展

肾母细胞瘤是儿童中最常见的实体瘤之一,其基因表达与肿瘤的发生、发展及预后密切相关,目前治疗方案不断改进,包括术前化疗、保留肾单位手术及肿瘤干细胞的靶向治疗,旨在维持较高生存率的同时减少治疗相关毒性.该文就肾母细胞瘤基因表达和治疗措施作一综述.     

小儿恶性肿瘤介入治疗进展

常用的肿瘤介入治疗包括经导管的动脉化疗栓塞(TACE)和经导管的动脉灌注化疗(TAIC或IAC)。TACE是将化疗药与栓塞剂注入肿瘤供血动脉,适用于肝、肾肿瘤。TAIC用于其他部位的肿瘤,只注入化疗药物而不用栓塞剂,进行局部的动脉大剂量冲击化疗,而避免邻近脏器受到栓塞。介入治疗所用的化疗药物有卡铂或顺铂,多柔比星或表柔比星,5-氟尿嘧啶,长春新碱或长春地辛等。栓塞剂有碘油、明胶海绵颗粒、微球颗粒等。根据肿瘤的状况,介入治疗可间隔4周重复1～3次,直至估计手术能完整切除肿瘤。国内TACE应用最多的儿童肿瘤为肝母细胞瘤和晚期肾母细胞瘤。TAIC被用于盆腔、卵巢、阴道、膀胱、骶尾部、腹腔、大网膜、肠系膜根部、臀部、上臂等部位恶性肿瘤的治疗。介入治疗与静脉用药全身化疗的交替应用具有更好的治疗作用。     

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Tumors of the salivary gland are very uncommon in children. Sialoblastoma is a rare, aggressive, blastomatous, and potentially malignant congenital tumor. Distant metastases are rare. We present a case of sialoblastoma with lung metastases that developed in a 4-year-old girl adjacent to a congenital nevus in the left cheek. The tumor was inoperable at diagnosis but the largest of the pulmonary metastases was removed surgically. The patient responded well to chemotherapy and underwent surgical excision of the primary tumor, followed by three more courses of chemotherapy.     

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目的观察全身应用卡铂、替尼泊苷和长春新碱(CTV)方案化疗加鞘内注射治疗脑脊液细胞学检查肿瘤细胞阳性的视网膜母细胞瘤(RB)患儿的疗效。方法6例患儿资料来自首都医科大学附属北京同仁医院2006年11月至2008年7月临床确诊为RB且治疗前行脑脊液细胞学检查发现肿瘤细胞者,对其进行6～9次CTV方案化疗加8～10次鞘内注射治疗,同时结合局部治疗,随访12～20个月,观察疗效。结果6例患儿均完成治疗,病情稳定,复查脑脊液细胞学检查均未发现肿瘤细胞,无复发及死亡。结论全身化疗加鞘内注射是对RB颅内转移有效的治疗方法,由于观察时间较短,长期疗效尚须行观察。     

The chemotherapy of granulosa cell tumors of the ovary: Experience of the wisconsin clinical cancer center

We reviewed the clinical records of 32 patients with ganulosa cell tumor of the ovary treated at the Wisconsin Clinical Cancer Center (WCCC) between 1970 and 1982. Eleven of these patients were treated with one or more chemotherapeutic regimens, yielding a total of 22 treatment trials. Objective response was observed in 7 of 17 evaluable treatment trials (41%). The response to chemotherapy could not be assessed in five treatment trials due to the concomitant administration of radiotherapy. We conclude that granulosa cell tumor of the ovary is responsive to chemotherapy. However, the optimal chemotherapeutic regimen for this rare neoplasm remains to be established on the basis of prospective clinical trials.     

Pancreatoblastoma: Case report and review of treatment in the literature

A case of pancreatoblastoma arising from the body-to-tail of the pancreas in a 5-year-old boy is presented. The patient underwent exploratory laparotomy and, 11 days later, resection of the tumor (partial pancreatectomy, pyloroplasty, and splenectomy). Before resection, cyclophosphamide and vincristine were administered. Because of tumor spillage during resection, a combination of chemotherapy (administration of cyclophosphamide and adriamycin on that day) and postoperative radiotherapy was given. Nine months after resection, partial hepatectomy was performed for liver metastasis and consolidated by a more intensive chemotherapy regimen using cisplatin, adriamycin, vincristine, and cyclophosphamide. After completion of the chemotherapy, the patient had a 14-month uneventful course, and a locally recurrent tumor was treated by the fourth surgery (extirpation of the recurrent tumor, partial hepatectomy, partial colectomy, and partial gastrectomy) and intraoperative radiation. Thereafter, the boy has shown no evidence of disease at 3 years 8 months. The literature of pancreatoblastoma is reviewed from the therapeutic viewpoint. © 1996 Wiley-Liss, Inc.     

Veno-occlusive disease of the liver in a child after chemotherapy for brain tumor

Veno-occlusive disease (VOD) of the liver is a life-threatening state generally occurring as a complication of bone marrow transplantation or chemotherapy for Wilms' tumor. Veno-occlusive disease after standard dose chemotherapy in malignancies other than Wilms' tumor is rare and only a few cases have been published in children. We report a 19 month-old-girl with medulloblastoma who experienced fatal VOD of liver after only one course of chemotherapy including carboplatin, vincristine and CCNU for medulloblastoma. As our knowledge, this is the first report of VOD after standard dose chemotherapy for brain tumor in childhood.     

Chemotherapy-Induced Histologic Changes in Wilms' Tumors

The nephrectomy specimens of 21 patients with Wilms' tumor who received preoperative chemotherapy consisting of vincristine and actinomycin D, alone or in combination, were studied pathologically; 1 patient also received radiation therapy. Pathological material from 20 randomly selected patients with Wilms' tumor who did not receive preoperative chemotherapy was reviewed and used as a control. Twenty tumors were of favorable histology, and one was unfavorable; all control tumors were of favorable histology. The histological changes were diffuse. The most marked changes occurred in the undifferentiated stroma of 18 tumors: the stroma was edematous; had a fibrovascular background, granulation tissue, and histiocytes; and lacked atypical cells. The blastematous nodules were reduced in size and necrotic or undergoing necrosis in 5 cases. Differentiated elements, including glomeruloid, tubular, and rhabdomyoblastic components, were unaffected. Vascular changes, consisting of fibrinoid necrosis, thrombosis, and acute inflammation were prominent in 15 instances. Two tumors of favorable histology, including one that was a rhabdomyomatous Wilms' tumor, and the tumor of unfavorable histology were unchanged by the therapy. In contrast, the histological changes present in only 4 of the control group, while similar, were rather minimal and focal. This evaluation helps to define the susceptibility of the different elements in Wilms' tumor to chemotherapy.     

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.     

Treatment of malignant testicular tumors in childhood: Results of the german national study 1982–1992

The German Cooperative Protocol for treatment of testicular germ cell tumors in childhood registered 106 patients from January 1982 through February 1992. Sixty-one patients suffered from yolk sac tumors (YST); 25 patients from differentiated teratomas (TD); 19 patients from malignant teratomas of either intermediate (MTI), undifferentiated (MTU), or trophoblastic type (MTT), and 1 patient from a seminoma. A stratified chemotherapy based on stage and histology was administered in addition to unilateral orchiectomy: Standard chemotherapy consisted of four treatments with vinblastine, bleomycin, and cisplatinum. If viable tumor was suspected after two treatments with standard chemotherapy, a delayed explorative laparotomy was done. There were two options based on the histological findings: In case of complete tumor regression, the standard chemotherapy was continued. In case of incomplete tumor response, patients a salvage chemotherapy consisting of three treatments with VP 16 (etoposide), ifosfamide, and cisplatinum. In addition three injections with VP 16 were given as a maintenance therapy. The following results were obtained: YST: 59 patients with stage I. Forty-nine patients were followed according to “wait and see” policy. Eight of these needed a delayed standard chemotherapy. The relapse free survival of all 61 patients in 100%. Median observation time is 49 months. TD: Twenty-five patients had stage I. No chemotherapy was given. The relapse free survival is 100%. Median observation time is 48 months. Malignant teratomas (MTI, MTU, MTT): 8 patients had stage I. Three of these adjuvant chemotherapy and 5 lymphadenectomy without chemotherapy. All patients survived without relapse. Nine patients had stage II and standard chemotherapy. Four of these patients had a delayed explorative laparotomy leading to a salvage therapy in two patients. All patients survived relapse free. Two patients had stage III. Of these 1 standard chemotherapy and is well. One patient suffering from MTU stage IIIA died due to candida septicemia during salvage therapy. Median observation time of the entire group is 60 months. © 1994 Wiley-Liss, Inc.     

The treatment of malignant scala posterior tumors in children: II. Preliminary result of the pre- and postoperative adjuvant chemotherapy of scala posterior tumors

Ten children with posterior scala tumor infiltrating the surrounding brain substance and/or the brain stem entered in the present study with preoperative chemotherapy. In 8 of the 10 cases regression and necrosis of the tumor were seen by CT examination after the preoperative therapy. The diameter of the tumor decreased on the average by 35.6% (14.0–74.3%). The main side effect was granulocytopenia. According to our observation, the preoperative therapy enables a more radical surgery in some cases of medulloblastoma and ependymoma. Further observations are necessary to confirm these preliminary results. © 1993 Wiley-Liss, Inc.     

Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy

Cystic partially differentiated nephroblastoma (CPDN) has low malignant potential. We report a 1-year-old with stage III CPDN of the right kidney that recurred following radical nephrectomy and chemotherapy. There was evidence of tumor spillage pre-operatively and intra-operatively. During chemotherapy the disease recurred in the omentum and the peritoneum. Pathology of the recurrent resected cysts revealed a more differentiated biphasic tumor without blastemal elements. It appears that spillage of CPDN in our patient led to dissemination of disease. Chemotherapy failed to prevent recurrence but only mature elements were present following this treatment. The intensity of therapy required to treat CPDN remains undefined.