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.     

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。     

术前介入在肝母细胞瘤治疗中的应用评价

目的：探讨介入治疗在小儿肝母细胞瘤综合治疗中的地位及适应证。方法：对估计不能切除的肿瘤施以碘油化疗药物栓塞，然后再外科手术，9例中有7例施行了肝叶切除，右三叶3例，右半肝2例，左半肝2例。结果：介入后，小儿副反应轻，肿瘤体积缩小，6例AFP值下降，未施手术1例介入后肺转移死亡，探查术1例后改肝移植术后死亡，肝叶切除7例中，死亡5例（3例复发，1例脑转移，1例肝功能衰竭）存活2例健在。结论：介治疗可以使某些不能Ⅰ期切除的肝母细胞瘤有了手术切除的机会，但要掌握适应证和方法。     

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。     

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。     

小儿肝母细胞瘤的诊断与治疗

现根据北美和欧洲肝母细胞瘤研究经验结合我国的临床实践,着重讨论肝母细胞瘤的诊断路径及治疗策略,以期提高对肝母细胞瘤的治疗水平.     

经导管动脉栓塞治疗婴儿颌面部巨大血管瘤

目的 评价婴儿颌面部巨大血管瘤,行经导管动脉栓塞治疗的疗效及安全性.方法 选择2005年至2007年间我院25例婴儿颌面部巨大血管瘤连续病例,行经股动脉插管血管瘤硬化栓塞(TASE)治疗,血管瘤供血动脉插管依次使用碘油博来霉素乳剂及聚乙烯醇泡沫颗粒栓塞.结果 25例共行TASE术35次,每例行1～3次,平均1.4次.平均住院3.2 d(2～5 d).25例中达治愈标准19例(76.0%),达显效标准6例(24.0%),无效0例,显效率100%,治疗结束后平均随访10.4个月,最长16个月,最短6个月,无复发病例.本组未出现肺栓塞、脑栓塞等严重并发症.结论 婴儿巨大血管瘤经导管动脉硬化栓塞治疗具有创伤小、安全、住院时间短、疗效显著、大多数病例术后不留瘢痕等优点.     

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］     

经导管动脉栓塞治疗婴儿颌面部巨大血管瘤

Objective To evaluate the therapeutic effects and safety of transcatheter arterial em-bolization (TAE) in infantile huge hemangiomas. Methods From 2005 to 2007, 25 consecutive cases of huge hemangioma that aged 1- 9mon ( mean 3.7±1.34 mon) and weighed 4.3 to 8kg (mean 6.52 ±0.86 kg) were treated by TAE. The support artery of hemangioma was embolized by Bleomycin-lip-iodol emulsion (PLE) and Polyvinyl Alcohol (PVA) in succession. Results Twenty-five cases under-went 35 times of TAE (1-3 times per each case with a mean of 1.4 times). The average hospitalization was 3.2 days (range 2-5 d ). The effective rate was 100% (25/25). The average follow-up period was 10. 44 months (range 6-16 months) without relapse. No serious complications such as pulmonary em-bolism and cerebral embolism occurred. Conclusions The advantages of TAE in the treatment for huge hernangioma in infancy are safe , effective, less trauma, short hospitalization and good cosmetic out-come.     

经导管动脉栓塞治疗婴儿颌面部巨大血管瘤

Objective To evaluate the therapeutic effects and safety of transcatheter arterial em-bolization (TAE) in infantile huge hemangiomas. Methods From 2005 to 2007, 25 consecutive cases of huge hemangioma that aged 1- 9mon ( mean 3.7±1.34 mon) and weighed 4.3 to 8kg (mean 6.52 ±0.86 kg) were treated by TAE. The support artery of hemangioma was embolized by Bleomycin-lip-iodol emulsion (PLE) and Polyvinyl Alcohol (PVA) in succession. Results Twenty-five cases under-went 35 times of TAE (1-3 times per each case with a mean of 1.4 times). The average hospitalization was 3.2 days (range 2-5 d ). The effective rate was 100% (25/25). The average follow-up period was 10. 44 months (range 6-16 months) without relapse. No serious complications such as pulmonary em-bolism and cerebral embolism occurred. Conclusions The advantages of TAE in the treatment for huge hernangioma in infancy are safe , effective, less trauma, short hospitalization and good cosmetic out-come.     

Hepatic arterial chemoembolization as an initial treatment for hepatoblastoma

Hepatic arterial chemoembolization using Lipiodol Ultra-fluid (Laboratoire Guerbet), doxorubicin HCl, and Gelfoam (Upjohn)(L-TAE) produced good results in an infant with a hepatoblastoma initially considered to be difficult to resect. Lipiodol selectively accumulated in the tumor, and a marked reduction in tumor size with decrease of alpha-fetoprotein was observed. One month after L-TAE, left hepatectomy was performed uneventfully with a good postoperative course. L-TAE appears to be a useful treatment for hepatoblastoma. Offprint requests to: I. Yamagiwa     

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.     

P-糖蛋白、多药耐药相关蛋白1、肺耐药相关蛋白和胎盘型谷胱甘肽-S-转移酶在肝母细胞瘤中表达特点及临床意义

目的探讨4种耐药基因蛋白P-糖蛋白（P-gp）、多药耐药相关蛋白1（MRP1）、肺耐药相关蛋白（LRP）和胎盘型谷胱甘肽-S转移酶（GST-π）在肝母细胞瘤中的表达特点与肝母细胞瘤病理类型的关系。方法采用免疫组织化学SP法检测20例肝母细胞瘤中P-gp、MRP1、LRP和GST-π的表达强度和分布。结果肝母细胞瘤具有原发耐药性，P-gp和LRP在肝母细胞瘤的多药耐药机制中起主要作用，MRP1起补充作用，GST-π起协同作用；4种耐药基因蛋白在肝母细胞瘤的表达与标本来源无关（P〉0．05）；P—gp在肝母细胞瘤不同病理类型中呈高强度强阳性表达。结论P—gP、MRP1、LRP和GST-π在肝母细胞瘤中表达的差异性，有助于肝母细胞瘤预后判断以及新辅助化疗中个体化化疗方案的制定。     

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.     

Surgical treatment of hepatoblastoma in children

Hepatoblastoma is the most common liver malignancy in children. With rare exceptions, complete tumour resection is required to cure the patient. Radical tumour resection can be obtained either with standard partial hepatectomy or orthotopic liver transplantation. At present, the surgical approach to hepatoblastoma differs significantly between treatment groups in different parts of the world. Our aim was to review current surgical policy in hepatoblastoma. All aspects of surgery in hepatoblastoma are discussed, including biopsy, tumour resection principles, modern achievements in the field of liver surgery, and the indications and potential contraindications for liver transplantation. Every effort should be made to resect hepatoblastoma completely either by standard partial hepatectomy or by the use of liver transplantation in difficult or clearly unresectable cases.     

Hemoperitoneum as the presenting sign of hepatoblastoma in a newborn

Hepatoblastoma is rarely diagnosed in the neonatal period. We report a neonate in whom massive hemoperitoneum was the presenting sign of hepatoblastoma: tumor injury during vaginal delivery resulted in tumor spread and hemoperitoneum leading to conservative surgical treatment. The authors propose that in cases where intrauterine ultrasonographic findings suggest a hepatic mass, cesarean section should be considered in order to improve the prognosis in cases of congenital hepatoblastoma.     

缺氧诱导因子及其相关蛋白在肝母细胞瘤中的表达

目的 探讨缺氧诱导因子及其相关蛋白在肝母细胞瘤(hepatoblastoma,HB)中表达情况及其作用.方法 选择1998年至2009年入住我院并接受手术的肝母细胞瘤患儿及胆道闭锁患儿各30例,分为三组:肝母细胞瘤肿瘤组织、距肿瘤组织边缘5 cm以外的正常肝脏组织以及胆道闭锁活检肝组织,肿瘤组中以PRETEXT分期系统,将肿瘤组分为低分组(Ⅰ组+Ⅱ组,10例)和高分组(Ⅲ组+Ⅳ组,20例).采用免疫组织化学染色方法检测HIF-1α、HIF-2α、VEGF、GLUT-1、TGF-α在上述标本中的表达情况,并且比较各组的表达情况差异.结果 5种蛋白的阳性表达均主要表现为细胞质内棕黄色颗粒.肿瘤组HIF-1α、HIF-2a、VEGF、GLUT-1、TGF-a表达明显高于其他两组(P<0.01).肿瘤高分组中HIF-1α及TGF-α的表达水平高于低分组(P<0.05),而HIF-2α、VEGF、GLUT-1的表达两者无显著差别.结论 缺氧诱导因子及其相关蛋白在肝母细胞瘤中的表达均有增加,提示HIF可能在肝母细胞瘤的发生发展中起着重要作用,其中HIF-1α还可能是潜在的预后判断指标之一.     

Acute lymphoblastic leukemia and hepatoblastoma in a family

Acute lymphoblastic leukemia is the most common and hepatoblastoma is a rare malignancy diagnosed in children. Their report on an Iranian boy with acute lymphoblastic leukemia diagnosed at the age of 2 years; 20 months later his 10-month-old sister was referred to their hospital with hepatoblastoma. The occurrence of such two types of cancer is rare in a family.