肝动脉栓塞化疗及延期手术治疗小儿切除困难的肝母细胞瘤

自2003年1月至2005年2月我科共收治小儿肝母细胞瘤8例，其中5例经B超，CT或MRI等检查临床诊断为ⅢA期肝母细胞瘤，无法Ⅰ期手术完整切除，行肝动脉栓塞化疗（TACE）疗程后均能完整切除肿瘤，无手术死亡病例，存活最长已2年，平均存活时间11个月，无肿瘤复发或转移。     

经导管动脉化疗栓塞在肝母细胞瘤综合治疗中的应用评价及文献回顾

目的：探讨经导管动脉化疗栓塞术（TACE ）在肝母细胞瘤综合治疗中的作用及疗效。方法回顾性总结2005年-2013年收治的10例经TACE 治疗的肝母细胞瘤患儿的临床资料。随访治疗后患儿的全身情况、血AFP值及瘤体体积大小的变化及生存情况。结果患儿10例,男女比例4∶1,发病中位年龄9.5个月（1个月～10岁）,10例患儿首诊后均予以1～4次TACE 治疗,其中6例TACE＋手术＋化疗,1例TACE＋化疗,3例在TACE 治疗后放弃继续治疗。介入治疗后肿瘤体积较前均有明显缩减（26.2％～10.00％,平均70.0％）,血清AFP值下降明显（28.5％～99.7％,平均83.6％）。所有患儿在接受TACE 治疗后均出现不同程度的发热、呕吐、一过性肝功能损害（Ⅰ度3例,Ⅱ度2例,Ⅲ度1例）、轻度骨髓抑制、贫血等不适,未见明显心脏毒性及肾毒性损害。随访时间2～114个月,平均随访时间为36.1个月,1年存活率100％（7／7）,2年存活率86％（6／7）,3年存活率71％（5／7）。6例患儿在接受TACE治疗后成功行手术切除,术后均接受全身化疗,1例仅经过4次TACE治疗及化疗后肿瘤消失,未行手术治疗,均无瘤存活至今。3例放弃治疗患儿分别于2～8个月后死亡。结论 TACE治疗可作为肝母细胞瘤术前重要的辅助治疗方式,能够使肿瘤体积明显缩小,血供减少,促进肿瘤包膜增厚,为尽可能完整切除创造了条件,能够改善肝母细胞瘤患儿的生活质量,提高长期存活率。     

不可切除型肝母细胞瘤的术前介入治疗临床研究

目的探讨术前介入性动脉栓塞化疗（TACE）在不可切除型肝母细胞瘤治疗中的可行性。方法分析对8例经检查诊断为不可切除型肝母细胞瘤行TACE的治疗结果。结果8例患儿TACE后均有不同程度呕吐、发热、一过性肝功能损害，1例有骨髓抑制，无心、肾功能损害。有6例复查B超检查，肿瘤体积较前均有明显缩小，缩小比例为22．5％～57．5％；5例手术完整切除，均已无瘤存活1～4年，3例复诊检查发现有肺转移或多发转移，家属放弃进一步治疗后死亡。结论TACE具有化疗药物剂量小、全身副作用少、化疗效果好的优点，可作为一种术前治疗方法应用于不可切除型肝母细胞瘤。     

经导管动脉化疗栓塞联合手术治疗儿童肝母细胞瘤

目的 探讨对不能切除的小儿肝母细胞瘤实行动脉化疗栓塞术(TACE)治疗的临床可行性、疗效及作用.方法 8例常规不能切除的肝母细胞瘤经TACE治疗20次,再行Ⅱ期外科手术切除,比较分析治疗前后临床症状、体征和血AFP的变化、术中情况及随访远期疗效.结果 TACE术后4～6周复查,和术前相比.肿瘤体积缩小67.68%～95.91%,平均缩小87.91%(t=3.754,P=0.007),血AFP下降94.46%～99.97%,平均98.45%(t=2.931,P=0.022),无明显的化疗毒性反应,所有患儿手术完整切除肿瘤,术后病理显示肿瘤明显坏死.随访8～24个月,所有患儿均获无瘤生存.结论 TACE治疗肝母细胞瘤安全有效,无严重并发症,能使部分不能Ⅰ期手术的患儿重新获得手术机会,并对减少术中肿瘤细胞的扩散和术中出血、改善预后有较大作用,可作为无远处转移肝母细胞瘤的术前辅助治疗.     

介入栓塞治疗后手术切除肝母细胞瘤

目的 评价介入栓塞对肝母细胞瘤的手术切除率及预后的影响.方法 回顾性分析2006年1月至2011年12月介入栓塞后经手术治疗的肝母细胞瘤33例患儿的临床资料.本组术前予介入栓塞治疗2～4个疗程后,均可手术切除,术后2周均予辅助化疗.结果 所有病例均获得肿瘤的完整切除,手术效果良好,术后随访6个月～5年,存活30例.结论 术前介入栓塞治疗可减少肿瘤血供,缩小肿瘤体积,提高手术切除率,综合治疗后可提高生存率.     

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。     

肝母细胞瘤的综合治疗及影响预后的因素评价

目的 探讨肝母细胞瘤的综合治疗方法,分析影响肝母细胞瘤治疗效果及生存率的因素.方法 对本院2000年1月-2007年11月收治的16例肝母细胞瘤患儿进行回顾性分析和随访,治疗以手术加化疗为主,化疗分为术前化疗和术后化疗,术前化疗11例又分为介入化疗5例和全身化疗6例(其中2例同时进行介入化疗和全身化疗);未进行术前化疗5例.行根治性手术治疗者9例,行姑息性手术治疗者3例,单纯静脉化疗者2例,单纯介入性肝动脉化疗栓塞(TACE)治疗1例,放弃治疗自动出院1例.随访时间13.5(3～98)个月.采用SPSS 13.0软件进行统计学分析.结果 Ⅳ期高危组患儿总体生存率明显较Ⅰ、Ⅱ、Ⅲ期标准组低;血清甲胎蛋白(AFP)<100μg/L和AFP>100000μg/L患者生存率低;混合型预后较好,而胚胎型及巨梁型预后较差;规范化疗组患者生存率明显高于不规范化疗组;手术组患者生存率明显高于非手术组:有残留组患者预后较无残留组差.结论 化疗可提高肝母细胞瘤中晚期患者的总体生存率和长期生存率,TACE是肝母细胞瘤患者一种安全有效的治疗方法;应提高对肝母细胞瘤的认识,及早发现,及时治疗.     

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。     

婴儿腹腔实体肿瘤临床病理与预后分析

目的探讨婴儿腹腔实体肿瘤的临床病理与预后的关系。方法回顾上海儿童医学中心1998年4月-2007年2月及上海新华医院2001年1月-2007年2月住院的52例1d～1岁腹部肿瘤患儿临床资料。患儿病史资料通过病史采集方式获得。病理类型通过复查病理切片和病理报告核对。随访采用电话和信访方式及患儿来院复诊记录。随访时间5个月～8a。结果52例中年龄1d～1个月7例,≥1个月～1岁45例。其中畸胎瘤23例(44.23%),神经母细胞瘤9例(17.31%),肾母细胞瘤6例(11.54%),肝母细胞瘤5例(9.62%),肝血管内皮瘤、先天性中胚层肾瘤各3例(5.77%),胰头梭形细胞血管内皮瘤、肝错构瘤、后腹膜小细胞恶性肿瘤各1例(1.92%)。良性肿瘤:恶性肿瘤=1:1,良性肿瘤中男:女=1:1,恶性肿瘤中男:女=2.33:1.0。患儿均手术切除肿瘤,恶性肿瘤辅以化疗。良性肿瘤均无复发;恶性肿瘤中复发或转移2例,其中1例死亡。失访4例。结论婴儿腹腔实体肿瘤以畸胎瘤和神经母细胞瘤居多。良性肿瘤数量与恶性肿瘤相当。良性发病无性别差异,手术切除后预后良好。恶性肿瘤中,男性比例显著高于女性,完整手术切除配合术后化疗,复发少,病死率低。早期诊断和治疗对其预后至关重要。     

新生儿和小婴儿肝脏婴儿型血管内皮瘤的诊断治疗探讨

目的　探讨新生儿和小婴儿的肝脏婴儿型血管内皮瘤的诊断治疗特点。方法　回顾分析 6例肝脏婴儿型血管内皮瘤并结合文献分析其临床表现、影像学检查、病理特点、治疗方法和疗效。结果　 6例中产前B超检查发现肝脏肿块 3例 ,因肝大腹部包块就诊 2例、黄疸 1例。无充血性心衰和Kasabach Merrit综合征等并发症发生。影像学检查 :B超均示肝脏低回声实性肿瘤影 ,血流丰富。CT平扫均示肝脏低密度肿块 ,可有散在或聚集颗粒状钙化 ;增强扫描边缘明显强化 ,逐渐向中心扩张。肿块大小不等。 6例均为单发病变 :肝右叶 4例、左叶 2例 ;肝内 2例、边缘 4例。血甲胎蛋白 (AFP)除 1例未查 ,5例均高于 12 10ng/mL。手术切除 5例 ,1例保守观察。病理特点 :6例均为Ⅰ型血管内皮瘤 :光镜下见由大小不等血管构成 ,管腔内壁可见肿胀增生的内皮细胞 ,核分裂相少见 ;免疫组化凝血Ⅷ因子 ( ) ,CD34( )。随诊 2个月至 2年 :手术患儿无复发 ,3例满 1岁者AFP降至正常 ;1例保守观察者随访至生后 7个月 ,肿瘤略缩小。 6例患儿均生长发育良好。结论　肝脏婴儿型血管内皮瘤发病早 ,多在胎儿晚期、新生儿期及 6个月内发现 ,为良性病变 ,须与肝母细胞瘤和神经母细胞瘤肝转移相鉴别 ;单发病灶无并发症者手术切除效果好 ,无症状者可以保守观     

Cure of hepatoblastoma with transcatheter arterial chemoembolization

Until now surgical resection was still considered as the only choice of successful treatment of hepatoblastoma. Therefore, successful use of transcatheter arterial chemoembolization (TACE) alone to cure the unresectable hepatoblastoma in an infant was firstly reported. A 6-month-old boy presented with a huge abdominal mass and was found to have a hepatoblastoma of 17.5 cm x 11.5 cm x 10 cm on computed tomography (CT) scan. The serum alpha-fetoprotein (AFP) was elevated to 6250 ng/mL. On the first TACE the main feeding arteries were completely occluded by stainless steel embolization coils. After one month tumor shrinkage was 75%, but a newly formed feeding artery was found and embolized on second TACE. Since the third TACE no newly formed feeding artery was found and 6 courses of intravenous chemotherapy were consolidated. On the last DSA and CT the tumor was completely disappeared and AFP returned to normal. During the follow-up he remained disease-free for 33 months until the present report. TACE may provide an additional promising choice in the treatment of hepatoblastoma.     

Combination chemotherapy and chemoembolization in the treatment of primary inoperable hepatoblastoma

A 33 months old girl and a 5 months old boy presented with unresectable hepatoblastoma (group III) and were treated with combination chemotherapy using Adriamycin, Cyclophosphamide, Cisplatinum (regimen I) and Adriamycin, Etoposide, Cisplatinum (regimen II). In both patients tumor size and alpha-fetoprotein levels decreased remarkably. Tumor regression was considerably enhanced by selective chemoembolization (Ethibloc, Adriamycin) of the right hepatic artery in one patient. After preoperative chemotherapy hepatoblastomas could be removed surgically. Local recurrence in the older patient was treated again successfully by surgery and chemotherapy. The patients are surviving disease-free 17 and 28 months from diagnosis. The efficacy of the treatment warrants prospective clinical trials in patients with unresectable hepatoblastoma.     

Combined treatment of hepatoblastoma with transcatheter arterial chemoembolization and surgery

Hepatoblastoma (HB) frequently presents at an advanced and unresectable stage. Transcatheter arterial chemoembolization (TACE) had been attempted to improve the feasibility of removing the bulky tumor in the authors' hospital and the results were presented here to evaluate the effectiveness and therapeutic role of TACE in the HB infants. Eight patients (6 boys, 2 girls), ranging in age from 2 months to 12 months, had unresectable HB based on clinical manifestation, B-ultrasound (B-US), chest X-ray film, computed tomography (CT), blood chemistry, and serum alpha-fetoprotein (AFP), and were subjected to TACE 1-3 times. On each TACE, Adriamycin (ADR, 20 mg/m2), vincristine (VCR, 1.5 mg/m2), and cisplatin (CDDP, 40 mg/m2) dispersed in 5-10 mL lipiodol were infused into the tumor, and stainless-steel embolization coils were released into the main feeding artery until completely embolized. Then, all the patients were reexamined once a month. Digital subtractive angiography was performed and the therapeutic strategy of further TACE or surgery was individualized in terms of the changes of tumor stain and the newly forming feeding artery. Six children (75%) had a marked response after the first TACE and were judged as being surgically resectable, but one boy died of pneumonia just before the scheduled operation and another boy preferred further TACE. The other 2 patients had only a partial response and required further TACE before the operation. Thus 6 children eventually underwent complete surgical resection and 1 boy achieved successful disappearance of tumor after 3 episodes of TACE alone. Seven children had an excellent recovery and remained tumor-free for 15-49 months. The results indicate that TACE is an effective and useful preoperative therapeutic choice for unresectable hepatoblastoma, and can improve the resectablity of the bulky tumor and the survival rate of HB patients. Multiple TACE could enhance the therapeutic effect and should be considered if indicated.     

Living-related liver transplantation with removal of inferior vena cava for unresectable hepatoblastoma

We report a case of a two-yr-old boy with hepatoblastoma resectable only by total hepatectomy including the vena cava. Successful LTx was performed with a living donor segment without vena cava reconstruction. The tumor was located in the bilateral lobe, surrounding the IVC. In spite of the high-dose chemotherapy, the tumor did not become resectable. LTx was performed using left lateral segment after removal of the IVC combined with total hepatectomy. Because the collaterals were well developed, the patient tolerated the procedure well. The serum AFP level decreased from 186 699 to 8 ng/mL in 11 months after LTx without local recurrence or distant metastasis.     

Prolonged survival following multiple thoracotomies for metastatic hepatoblastoma

A 14-month-old boy presented with hepatoblastoma, which was completely excised. He had pulmonary metastases, diagnosed 1 year later, treated with chemotherapy followed by resection at age 2 1/2 years. At the age of 3 1/2 years a further metastasis was shown on CT scan and this was resected. Two further metastses were resected at ages 4 1/2 and 5 1/2 years. At age 9 1/2 he had a further isolated pulmonary metastasis resected and is again disease-free more than 30 months from the last thoracotomy. Prolonged survival following metastatic hepatoblastoma can be achieved with repeated thoracotomies, especially if the first recurrence is more than 6 months from diagnosis and the number of metastases is small. Follow-up examinations should be both radiological and with serum alpha fetaprotein (SαFP). © 1995 Wiley-Liss, Inc.     

14例儿童肝母细胞瘤综合治疗的疗效观察

目的：肝母细胞瘤是儿童时期最为常见的原发性肝脏恶性肿瘤。新辅助化疗﹢手术﹢术后化疗已成为肝母细胞瘤治疗的基本原则。该文旨在总结肝母细胞瘤综合治疗的疗效,进而探讨合理治疗的策略。方法：回顾性分析14例儿童肝母细胞瘤患者的临床资料,并追踪随访其治疗后的生存状况。结果：12例肝母细胞瘤患儿接受全程治疗,中位随访时间为18个月（1.5~74月）。9例无瘤存活,1例死亡,1例肿瘤转移,1例未发现肿瘤残留但术后甲胎蛋白持续不降。结论：手术及规范化疗能有效提高儿童肝母细胞瘤患者的生存率。［中国当代儿科杂志,2009,11（6）：456-459］     

Preoperative chemoembolization for unresectable hepatoblastoma

 Complete surgical resection offers the only chance for cure in patients with hepatoblastoma (HB). Patients with unresectable lesions are given preoperative chemotherapy in an attempt to create a resectable lesion. We present a case of an 11-month-old with an unresectable stage III HB unresponsive to systemic chemotherapy. Transfemoral hepatic-artery chemoembolization resulted in a surgically resectable tumor. The patient underwent a right trisegmentectomy with complete resection of the tumor and remains tumor-free 24 months postoperatively. Salvage chemoembolization can be an effective preoperative modality to convert an unresectable tumor into a resectable one. Accepted: 19 January 2001