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］     

儿童晚期肝母细胞瘤综合治疗的疗效观察

目的 总结晚期肝母细胞瘤(HB)患儿的临床治疗结果,探讨晚期HB的临床疗效及诊疗经验.方法 回顾性分析2006年4月至2012年8月北京同仁医院儿科收治的40例经病理确诊及分期明确的晚期HB患儿(男29例,女11例;发病年龄3个月～11岁6个月,中位年龄1.9岁)的病例资料,根据美国儿童肿瘤组织肝母细胞瘤国际分期分为Ⅲ期15例,Ⅳ期25例,采用多科室协作模式进行治疗.结果 随访至2012年8月,中位随访时间为21个月(1 ～ 67个月),失访1例,余39例患儿获随访,死亡12例(其中1例为进展期放弃治疗死亡,1例化疗中合并脏器衰竭死亡),完全缓解及部分缓解共27例(其中1例为进展期放弃治疗至随访日仍存活),总生存率为69.2％(27/39例).结论 晚期HB儿童一旦伴有远处组织器官转移预后较差,但综合治疗仍可延长患儿生存期及提高临床缓解率.     

新生儿肝母细胞瘤1例

患儿 ,男 ,2 8ｄ ,因皮肤黄染 2 5ｄ入院。第 1胎 ,第 1产 ,足月顺产 ,新法接生 ,无窒息史。母乳喂养。生后 3ｄ发现皮肤黄染 ,且渐加重 ,尿黄 ,无陶土样大便 ,吃奶量较少 ,日渐消瘦 ,睡眠多。体检 :体温 37.4℃ ,体重 2 .5ｋｇ。神志清 ,精神不振 ,营养不良Ⅱ度 ,全身皮肤粘膜重度黄染 ,未见蜘蛛痣 ,浅表淋巴结无肿大 ,巩膜明显黄染 ,双肺呼吸音清 ,心率10 0次 /ｍｉｎ ,律齐 ,心音低钝 ,心尖区闻及Ⅱ～ⅢＳＭ/ 6级。腹软 ,未见肠型及蠕动波 ,无腹壁静脉曲张 ,肝肋下 7.6ｃｍ ,剑下 4.5ｃｍ ,质韧 ,表面不光滑 ,脾肋下 2ｃｍ ,表面光滑。无…     

儿童肝母细胞瘤研究现状

肝母细胞瘤(hepatoblastoma,HB)是儿童期最常见的肝脏恶性肿瘤。HB起病隐匿,诊断和治疗都有一定的困难。近年来HB的发病率有上升趋势,对HB的研究也逐渐深入。文章就HB的病因、病理分型、临床分期、预后因素及治疗原则等研究结果作一综述。     

肝母细胞瘤的CT与临床表现：附11例报告

肝母细胞瘤是儿童肝脏肿瘤中最常见的肿瘤,但其发病率低,恶性程度高。现对我院1986年3月～1995年8月确诊的12例肝母细胞瘤。就其临床、CT及病理表现进行讨论如下。     

小儿“不能切除”的肝母细胞瘤的治疗

５例“不能切除”的肝母细胞瘤应用以顺铂为主的强效化疗后，肿瘤缩小，成功地施行了二期手术和延期手术，生存５－３０个月，提示化疗有效，但不同组织类型其疗效有差异。     

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.     

复发肝母细胞瘤的治疗进展

复发肝母细胞瘤依然是治疗的难点.本文介绍了肝母细胞瘤复发时的特点,包括复发部位、复发率、肿瘤复发相关的危险因素.同时重点介绍复发肿瘤的治疗方法,包括不同复发部位的肿瘤再手术、化疗、放疗、靶向治疗等,综述了这些治疗措施的临床应用或实验室研究进展,并分别比较了各种治疗方法的有效率.     

基于甲胎蛋白临床诊断肝母细胞瘤行新辅助化疗可行性分析

目的探讨基于血清甲胎蛋白(AFP)升高和肝脏影像学的临床诊断行经验性新辅助化学治疗在儿童肝母细胞瘤临床诊疗中的可行性。方法回顾分析近5年收治的80例原发肝占位患儿的临床资料,其中肝母细胞瘤50例和非肝母细胞瘤30例。分析血清AFP对临床诊断儿童原发肝占位为肝母细胞瘤的灵敏度和特异度;根据化疗前是否有病理证据将50例肝母细胞瘤患儿分为经验性化疗组和有病理化疗组,比较两组最终病理结果、总生存率(OS)和无事件生存率(EFS)。结果血清AFP异常升高诊断儿童原发肝占位为肝母细胞瘤的灵敏度为98%,特异度100%;与病理诊断的一致性高(Kappa=0.97,P0.001)。肝母细胞瘤经验性化疗组和有病理化疗组3年OS分别为88.3%和90.7%,EFS分别为74.8%和81.3%;经Log-rank检验,OS和EFS的差异均无统计学意义(P0.05)。经COX回归分析发现,在校正年龄、性别因素后,化疗前有无病理证据对PRETEX分期Ⅲ、Ⅳ期肝母细胞瘤3年OS和EFS影响无统计学意义(HR=4.68,95%CI:0.55～39.91;HR=2.34,95%CI:0.53～10.28)。结论根据血清AFP明显升高,可鉴别儿童肝母细胞瘤与其他儿童原发肝占位性疾病。基于血清AFP升高临床诊断肝母细胞瘤可指导手术耐受差的患儿术前新辅助化疗,降低手术风险。     

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.     

儿童初治肝母细胞瘤83例临床特征及预后分析

目的探讨肝母细胞瘤(HB)患儿的临床特征、生存情况及预后危险因素。方法回顾性分析2012年1月至2019年10月郑州大学第一附属医院儿童医院血液肿瘤科收治的83例初治HB患儿的临床资料, 记录患儿的性别、年龄、首发临床表现、治疗前病变范围(PRETEXT)分期、病理类型、病初甲胎蛋白(AFP)、治疗方法及治疗结果。2018年之前确诊的患儿采用"武汉方案"治疗, 2018年之后确诊的患儿采用"儿童肝母细胞瘤多学科诊疗专家共识(CCCG-HB-2016)"方案治疗。Kaplan-Meier生存分析法计算生存率, 单因素分析采用Log-Rank检验, 多因素预后分析采用Cox回归模型。结果 83例患儿中男51例、女32例;发病年龄25.2(9.0, 34.0)月龄, <3岁64例(77%)。最常见的首发临床表现为腹部包块(45例, 54%)。PRETEXT Ⅰ期8例, Ⅱ期43例, Ⅲ期20例, Ⅳ期12例。随访时间40(17, 63)个月, 全组HB患儿1年总体生存率(OS)和无事件生存率(EFS)分别为(84±4)%和(79±5)%, 5年OS和EFS分别为(78±5)%和(76±...     

Percutaneous radiofrequency ablation for treatment of hepatoblastoma recurrence

Radiofrequency ablation (RFA) has been widely reported as a minimally invasive treatment for liver tumours in adults, but has not been documented as a treatment for hepatoblastoma in a child. We report a 2-year-old boy with local recurrence of hepatoblastoma after partial hepatectomy. Percutaneous RFA was performed under real-time sonographic guidance. There was no imaging evidence of recurrence after a follow-up of 2 years. We consider this a promising technique in children.     

39例儿童头颈部横纹肌肉瘤临床特点及治疗转归

目的：探讨儿童头颈部横纹肌肉瘤的（RMS）临床特点及治疗转归。方法：回顾性分析北京同仁医院2004年11月至2010年11月收治的39例原发于头颈部的RMS患儿临床资料及治疗随访结果,其中男23例,女16例,年龄3个月至14岁,中位年龄6.0岁。结果：39例患儿临床主要表现为眼球突出及眼睑肿胀(56%,22/39),鼻塞及鼻出血占28%（11/39）,面颊包块占15%（6/39）。39例患儿中,原发部位以眼睑及眼眶为主（56%,22/39）,其次为鼻咽部及筛窦（28%,11/39）。37例有明确病理分型,大多数患儿为胚胎型（89%,33/37）。39例患儿中位随访时间为38个月(10~80个月),放弃治疗4例,获随访35例。35例患儿中,行单纯手术4例,单纯化疗1例, 手术+化疗12例,手术+放疗2 例,手术+化疗+放疗13例(其中8例行125I放射性粒子植入术治疗),手术+化疗+放疗+自体外周血造血干细胞移植（APBSCT）2例,化疗+APBSCT 1例。其中7例复发,5例死亡,总生存率86%（30/35）,完全缓解率达66%（23/35）,部分缓解率达20%（7/35）,8例粒子植入术治疗患儿6例达无瘤生存。结论：RMS患儿临床表现主要为眼球突出及眼睑肿胀;眼部及鼻咽是儿童头颈部RMS多发部位;病理分型以胚胎型为主;包括化疗、手术、125I粒子组织间植入及APBSCT等多种综合治疗方法可有效提高RMS患儿缓解率。     

儿童肝母细胞瘤肝组织中GRP78和GRP94表达及其临床病理意义

目的：研究肝母细胞瘤（HB）患儿肝组织中葡萄糖调节蛋白78和94 （GRP78和GRP94）表达水平及其临床病理意义。方法：15例儿童HB和10例正常肝组织标本常规制作石蜡包埋切片,用EnVsion免疫组织化学法,检测GRP78和GRP94表达。结果：HB患儿肝组织中GRP78和GRP94表达阳性率明显高于正常肝组织（53% vs 10%, 60% vs 10%; 均P0.05)。结论：GRP78和GRP94表达在儿童HB发生、进展方面可能有重要作用。［中国当代儿科杂志,2010,12（8）：634-636］     

Radiological staging in children with hepatoblastoma

Hepatoblastoma is the most common malignant liver tumour of childhood. Accurate radiological staging is very important, especially in children who are treated according to the protocols of the International Childhood Liver Tumor Strategy Group (SIOPEL). These protocols use risk stratification, based almost entirely on imaging findings, to minimize the treatment for localized tumours and to intensify treatment for extensive tumours and those with extrahepatic spread.     

Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7±0.3 cycles of chemotherapy before the operation. Right lobectomy (n=12), left lobectomy (n=5), extended left lobectomy (n=4), and extended right lobectomy (n=3) and right lobectomy with enucleation of two masses from left lobe (n=1) were performed. Intraoperative blood transfusion of 30.7±6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n=5), hepatocellular carcinoma (n=4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n=1), hemangioma (n=1), cyst adenoma (n=1), and metastasis of cellular mesoblastic nephroma (n=1). Patients were observed in the intensive care unit for 3.4±0.3 days. Postoperative complications were sepsis (n=1), disseminated intravascular coagulation (n=2), fever (n=3), jaundice (n=3), intraabdominal abscess (n=3), ileus (n=2), and subdiaphragmatic abscess with pleural effusion (n=1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control. An erratum to this article can be found at