Table CDR62836 Childhood Liver Cancer Treatment/儿童肝癌治疗
ID Type Description_en Description_zh
w1 Word Prognosis 预后
w2 Word C—Caudate C-尾叶
w3 Word Beckwith-Wiedemann Beckwith-Wiiedemann综合征
w4 Word Hepatoblastoma 肝母细胞瘤
w5 Word Tyrosinemia 酪氨酸血症
w6 Word Unusual 不常见
w7 Word Hepatocellular Carcinoma 肝细胞癌
w8 Word PRETEXT designations PRETEXT命名
w9 Word Recurrent Hepatoblastoma 肝母细胞瘤复发
w10 Word Transarterial chemoembolization 经肝动脉化疗栓塞术
w11 Word PRETEXT stage PRETEXT分期
w12 Word Vascular involvement 血管受累
w13 Word Stage III III期肿瘤
w14 Word Stage Information 分期信息
w15 Word AFP levels AFP水平
w16 Word Epithelioid Hemangioendothelioma 上皮样血管内皮细胞瘤
w17 Word Associated Disorder 相关疾病
w18 Word Stage IV IV期肿瘤
w19 Word Surgical Approaches 手术方式
w20 Word Alagille syndrome 先天性肝内胆管发育不良综合征（Alagille综合征）
w21 Word Presurgical chemotherapy 术前化疗
w22 Word Treatment-related factors 治疗相关因素
w23 Word Tumor Location 肿瘤部位
w24 Word Fibrolamellar carcinoma 纤维板层型肝癌
w25 Word Low-birth-weight infants 低出生体重儿
w26 Word General Information 基本信息
w27 Word Cellular Classification 细胞学分类
w28 Word M—Distant metastatic M-远处转移
w29 Word Biliary Obstruction 胆道梗阻
w30 Word E—Extrahepatic contiguous E-肝外毗邻部位浸润
w31 Word Stage explanation--legacy 分期信息--legacy
w32 Word childhood hepatoblastoma 儿童肝母细胞瘤
w33 Word No metastases 无转移
w34 Word Postsurgical chemotherapy 术后化疗
p1 Phrase PRETEXT stage 4 PRETEXT 4期
p2 Phrase PRETEXT stage 2 PRETEXT 2期
p3 Phrase PRETEXT stage 3 PRETEXT 3期
p4 Phrase Tumor marker–related factors 肿瘤标志物相关因素
p5 Phrase Childhood Rhabdomyosarcoma Treatment 儿童横纹肌肉瘤的治疗
p6 Phrase Recurrent Hepatocellular Carcinoma 肝细胞癌复发
p7 Phrase childhood liver cancer 儿童肝癌
p8 Phrase Other Treatment Approaches 其他治疗方法
p9 Phrase Biliary Tract Rhabdomyosarcoma 胆道横纹肌肉瘤
p10 Phrase Orthotopic liver transplantation 原位肝移植
p11 Phrase PRETEXT stage 1 PRETEXT 1期
p12 Phrase Age at Diagnosis 诊断年龄
p13 Phrase Familial adenomatous polyposis 家族性腺瘤性息肉病
p14 Phrase Treatment of Hepatoblastoma 肝母细胞瘤的治疗
p15 Phrase childhood hepatocellular carcinoma 儿童肝细胞癌
p16 Phrase Surgery and chemotherapy 手术与化疗
p17 Phrase Median age 3.4 y 中位数：3.4岁
p18 Phrase Beckwith-Wiedemann syndrome and hemihyperplasia Beckwith-Wiiedemann综合征与偏侧发育过度
p19 Phrase Stage IV (distant metastases) IV期（远处转移）
p20 Phrase Trisomy 18, other trisomies 18三体综合征或其他三体综合征
p21 Phrase Transitional liver cell tumor 过渡型肝细胞肿瘤
p22 Phrase Intratumoral injection of alcohol 肿瘤内注射无水乙醇
p23 Phrase Pure fetal histology hepatoblastoma 单纯胎儿型肝母细胞瘤
p24 Phrase Median age 10.5 y 中位数：10.5岁
p25 Phrase recurrent childhood liver cancer 儿童肝癌复发
p26 Phrase Chemotherapy and metastatic disease 化疗与肿瘤转移
p27 Phrase Hepatitis B and C 乙肝与丙肝
p28 Phrase Glycogen storage diseases I–IV I-IV型糖原储积症
p29 Phrase Small cell undifferentiated hepatoblastoma 小细胞未分化型肝母细胞瘤
p30 Phrase Hepatoblastoma and Hepatocellular Carcinoma 肝母细胞瘤与肝细胞癌
p31 Phrase Treatment of Hepatocellular Carcinoma 肝细胞癌的治疗
p32 Phrase Stages I and II I期与II期肿瘤
p33 Phrase Progressive familial intrahepatic cholestasis 进行性家族性肝内胆汁淤积症
p34 Phrase 25% for patients with metastasis 有转移癌的患儿：25%
p35 Phrase Adapted from Nicol et al 引用Nicol et al.
p36 Phrase 68% for PRETEXT stage 3 PRETEXT 3期：68%
p37 Phrase Treatment Options for Stage IV IV期肝母细胞瘤的治疗选择
p38 Phrase 57% for PRETEXT stage 4 PRETEXT 4期：57%
p39 Phrase Treatment options by cell type 不同细胞类型的治疗选择
p40 Phrase 22% for PRETEXT stage 3 PRETEXT 3期：22%
p41 Phrase Surgical resection for metastatic disease 转移灶的手术切除
p42 Phrase 61% for PRETEXT stage 4 PRETEXT 4期：61%
p43 Phrase Microscopic residual disease after resection 切除术后显微镜下残留病灶
p44 Phrase The chemotherapy was well tolerated 患儿对新辅助化疗的耐受性较好
p45 Phrase Limited Role for Radiation Therapy 放疗的有限作用
p46 Phrase Treatment Options for Stage III III期肝母细胞瘤的治疗选择
p47 Phrase Childhood Liver Cancer (PDQ®): Treatment 儿童肝癌（PDQ®）：治疗
p48 Phrase There are positive lymph nodes 淋巴结阳性
p49 Phrase 91% for PRETEXT stage 2 PRETEXT 2期：91%
p50 Phrase Infantile Choriocarcinoma of the Liver 婴儿肝绒毛膜癌
p51 Phrase Size of the primary tumor 原发肿瘤的大小
p52 Phrase 44% for PRETEXT stage 1 PRETEXT 1期：44%
p53 Phrase Hepatoblastoma prognosis by postsurgical stage 术后分期与肝母细胞瘤的预后
p54 Phrase 8% for PRETEXT stage 4 PRETEXT 4期：8%
p55 Phrase Presence of multifocal hepatic disease 是否存在多灶性肝病
p56 Phrase 95% for PRETEXT stage 2 PRETEXT 2期：95%
p57 Phrase 84% for PRETEXT stage 3 PRETEXT 3期：84%
p58 Phrase 44% for PRETEXT stage 2 PRETEXT 2期：44%
p59 Phrase Special considerations for surgical resection 手术切除的特殊考虑因素
p60 Phrase 100% for PRETEXT stage 1 PRETEXT 1期：100%
p61 Phrase Postsurgical Staging for Childhood Liver Cancer 儿童肝癌的术后分期
p62 Phrase P—Main portal or portal bifurcation vein P-主门静脉或门静脉分支静脉
p63 Phrase High risk Any patient with metastases 高危任何有转移的患者
p64 Phrase Treatment of Recurrent Childhood Liver Cancer 儿童肝癌复发的治疗
p65 Phrase Any stage may have involvement of: 各分期均可能有下列结构受累：
p66 Phrase Hepatitis B and hepatitis C infection 乙肝与丙肝病毒感染
p67 Phrase Cytotrophoblasts and syncytiotrophoblasts are both present 婴儿肝绒毛膜癌中可见细胞滋养层与合体滋养层细胞
p68 Phrase Undifferentiated Embryonal Sarcoma of the Liver 肝脏未分化胚胎性肉瘤
p69 Phrase Of these 11 patients, 9 survived 这11例患者中有9例存活
p70 Phrase Late Effects of Treatment for Childhood Cancer 儿童肿瘤治疗的后期效果
p71 Phrase Survival was 100% with no chemotherapy given 无化疗的情况下生存率达100%
p72 Phrase Chemotherapy followed by reassessment of surgical resectability 化疗后应重新评估肿瘤的可切除性
p73 Phrase Stages III and IV are usually fatal III期与IV期肝细胞癌通常致死
p74 Phrase Presurgical Staging for Hepatoblastoma and Hepatocellular Carcinoma 肝母细胞瘤和肝细胞癌的术前分期
p75 Phrase Treatment Options for Postsurgically Staged Stage IV 术后分期为IV期患者的治疗选择
p76 Phrase Treatment of Infantile Choriocarcinoma of the Liver 婴儿肝绒毛膜癌的治疗
p77 Phrase V—Vena cava or all three hepatic veins V-下腔静脉或所有三支肝静脉
p78 Phrase Several studies have tested different chemotherapy regimens 另几项研究比较了不同化疗方案的效果
p79 Phrase Treatment Options for Stages I and II I期与II期肝母细胞瘤的治疗选择
p80 Phrase Delayed surgical resection (chemotherapy followed by surgery) 延期手术（术前化疗）
p81 Phrase Frequently; jaundice is a common presenting symptom 多见，黄疸为常见症状
s1 Sentence Patients must be aged 2 to 30 years 患者年龄在2-30岁之间
s2 Sentence Initial surgical resection (alone or followed by chemotherapy) 早期手术（单独使用或联合术后化疗）
s3 Sentence AFP level must be greater than 100 ng/ml AFP水平必须大于100ng/mL
s4 Sentence Grossly resected disease with small cell undifferentiated elements 有小细胞未分化成分的大部切除肿瘤
s5 Sentence The timing of the surgical approach is critical 手术时机非常关键
s6 Sentence Surgery (usually biopsy only), radiation therapy, and chemotherapy 手术（通常仅活检）、放疗、化疗
s7 Sentence Often arises in the hilum of the liver 常见于肝门
s8 Sentence Combined vincristine/irinotecan has been used with some success 研究发现长春新碱/伊立替康联合化疗亦获得一定成功
s9 Sentence Treatment of Undifferentiated Embryonal Sarcoma of the Liver 肝脏未分化胚胎性肉瘤的治疗
s10 Sentence Treatment Options for Presurgically Staged (PRETEXT) Stage 4 术前分期（PRETEXT）为4期患者的治疗选择
s11 Sentence Intermediate risk Gross residual disease or unresectable disease 中危有肉眼残余病灶或肿瘤无法切除
s12 Sentence Prognosis varies depending on the subtype of hepatoblastoma: 其预后与肝母细胞瘤的亚型相关：
s13 Sentence This staging system is used to determine treatment 这一分期系统用于确定治疗选择
s14 Sentence A randomized clinical trial compared cisplatin/vincristine/fluorouracil with cisplatin/doxorubicin 一项随机临床试验比较了顺铂/长春新碱/氟尿嘧啶联合化疗和顺铂/阿霉素联合化疗对肝母细胞瘤的作用
s15 Sentence Hepatoblastoma and hepatocellular carcinoma prognosis by PRETEXT stage 肝母细胞瘤与肝细胞癌的PRETEXT分期与预后
s16 Sentence Chemotherapy followed by complete surgical resection of primary tumor 化疗后对原发肿瘤进行完全手术切除
s17 Sentence Response to chemotherapy is poor, much like hepatocellular carcinoma 和肝细胞癌类似，其对化疗反应较差
s18 Sentence Treatment options under clinical evaluation for recurrent hepatocellular carcinoma 处于临床评估阶段的复发性肝细胞癌治疗方案
s19 Sentence The lead reviewers for Childhood Liver Cancer Treatment are: 儿童肝癌治疗的首席审核人包括：
s20 Sentence Small cells that reflect neither epithelial nor stromal differentiation 非上皮或间质分化的小细胞型
s21 Sentence Often arises in the right lobe of the liver 常见于肝右叶
s22 Sentence In stage I hepatoblastoma, the tumor is completely resected I期肝母细胞瘤中，肿瘤被完整切除
s23 Sentence Complete surgical resection of primary tumor followed by chemotherapy 对原发肿瘤进行完全手术切除并辅以术后化疗
s24 Sentence Stage II is too rarely seen to predict outcome II期很少见，故很难以此预测预后
s25 Sentence Resection to be determined by POSTTEXT staging as above 根据前文所述POSTTEXT分期系统决定是否进行手术切除
s26 Sentence Treatment Options Under Clinical Evaluation: COG Hepatoblastoma Risk Groups 处于临床评估阶段的治疗选择：COG肝母细胞瘤危险分组
s27 Sentence Fibrolamellar hepatocellular carcinoma has also been reported in infants 亦有关于婴儿患纤维板层型肝癌的报道
s28 Sentence Occasionally neuronal, melanocytic, squamous, and enteroendocrine elements are found 有时则会出现神经元、黑色素细胞、鳞状细胞与神经内分泌成分
s29 Sentence Widespread infiltration throughout the liver and pulmonary metastasis are common 常见病灶弥漫性浸润全肝，肺转移多见
s30 Sentence Expert-reviewed information summary about the treatment of childhood liver cancer 关于儿童原发性肝癌治疗的专家审核信息总结
s31 Sentence Hepatocellular carcinoma prognosis by postsurgical stage of disease at diagnosis 术后分期与肝细胞癌的预后
s32 Sentence Infants are often unstable due to hemorrhage of the tumor 其出血常导致婴儿生命体征不稳定
s33 Sentence Children with stage I hepatocellular carcinoma have a good outcome I期的肝细胞癌患儿预后良好
s34 Sentence Hepatoblastoma is most often unifocal, and resection is often possible 肝母细胞瘤常呈单发病灶，多可被切除
s35 Sentence In stage II hepatoblastoma, microscopic residual tumor remains after resection II期肝母细胞瘤中，切除后仍有镜下残留肿瘤
s36 Sentence Identical overall results were seen in a follow-up international study 一项国际性随访研究结果与之一致
s37 Sentence Cure of hepatoblastoma or hepatocellular carcinoma requires gross tumor resection 肝母细胞瘤和肝细胞癌的治愈均需行肿瘤全切
s38 Sentence Table 1. Risk Factors Associated With Hepatoblastoma and Hepatocellular Carcinoma 表1. 与肝母细胞瘤与和肝细胞癌相关的危险因素
s39 Sentence The 10-year survival rates were similar to the 5-year rates 10年生存率和5年生存率类似
s40 Sentence The 5-year OS for PRETEXT staged hepatocellular carcinoma was as follows: 不同PRETEXT分期的肝细胞癌患者5年OS如下：
s41 Sentence An alternative treatment approach of transarterial chemoembolization for surgically unresectable disease 手术无法切除的肿瘤亦可以选择使用经肝脏动脉化疗栓塞术
s42 Sentence Patients whose tumors remain unresectable should be considered for liver transplantation 对于仍不可切除的肿瘤患者，应考虑行肝移植
s43 Sentence Maternal and infantile choriocarcinoma both come from the same placental malignancy 母亲与婴儿的绒毛膜癌均起源于胎盘恶性肿瘤
s44 Sentence There are two standard surgical staging systems for pediatric liver tumors 目前对儿科的肝脏肿瘤有两套标准的外科分期系统
s45 Sentence AFP is very high in neonates and steadily falls after birth 新生儿的AFP水平较高，出生后其水平才稳定回落
s46 Sentence The latter are very large multinucleated syncytia formed from the cytotrophoblasts 后者则是巨大的多核合胞体，来源于滋养层细胞
s47 Sentence Approximately 10% to 20% of children with hepatoblastoma are stage IV 约有10%-20%的肝母细胞瘤患儿分期为IV期
s48 Sentence Additional chemotherapy will follow if the primary tumor was completely resected 原发肿瘤完全切除后应继续进行化疗
s49 Sentence Most often the tumor consists of a mixture of epithelial hepatocyte precursors 最常见为混合上皮肝细胞前体型
s50 Sentence Chemotherapy followed by reassessment of surgical resectability followed by complete surgical resection 化疗，化疗后对手术可切除性进行重新评估，再进行完全手术切除
s51 Sentence (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.) （更多信息请参见PDQ总结：儿童横纹肌肉瘤的治疗）
s52 Sentence It may appear solid or cystic on imaging, frequently with central necrosis 肝脏未分化胚胎性肉瘤在影像学上表现为实性或囊性，常有中心坏死
s53 Sentence Orthotopic liver transplantation has been successful in selected children with hepatocellular carcinoma 原位肝移植已经成功用于治疗一些肝细胞癌患儿
s54 Sentence Addition of carboplatin to intensify the cisplatin/doxorubicin may have reduced its efficacy 使用卡铂强化顺铂/阿霉素方案或可降低其有效性
s55 Sentence Survival rates at 3 to 5 years range from 20% to 60% 3到5年的生存率在20%-60%
s56 Sentence Whether hepatic disease meets surgical and histopathologic criteria for orthotopic liver transplantation 肝病是否已达到原位肝移植的外科和组织病理学标准
s57 Sentence (refer to the Stage Information section of this summary for more information) （更多信息参见本总结分期信息一节）
s58 Sentence The tumors are usually unifocal and may have central necrosis at presentation 过渡型肝细胞肿瘤常为单发，中心多有坏死
s59 Sentence Often surgical removal of the residual primary tumor is performed after neoadjuvant chemotherapy 通常在新辅助化疗后再对原发肿瘤残留灶行手术切除
s60 Sentence Between 1975 and 2002, childhood cancer mortality has decreased by more than 50% 1975到2002年间，儿童癌症死亡率已下降50%
s61 Sentence Whether preoperative chemotherapy may convert an unresectable tumor into a potentially resectable tumor 术前化疗是否可将无法切除的肿瘤缩小成为可切除的肿瘤
s62 Sentence Both histologic types more commonly arise in the right lobe of the liver 两种组织学类型都常发生于肝右叶
s63 Sentence Chemotherapy can often decrease the size and extent of hepatoblastoma, allowing complete resection 化疗常可以缩小肝母细胞瘤的体积和范围，以使其可完全切除
s64 Sentence The prognosis for a patient with recurrent or progressive hepatocellular carcinoma is poor 肝细胞癌复发或进展的患者预后较差
s65 Sentence Liver transplant should be considered for patients with isolated recurrence in the liver 对于仅有孤立性肝复发灶的患者可以行肝移植术
s66 Sentence Any patient with initial AFP level less than 100 ng/ml, regardless of stage 初诊AFP水平低于100ng/mL，不论分期如何
s67 Sentence Distinctive features are intracellular hyaline globules and marked anaplasia on a mesenchymal background 其特点包括细胞内透明颗粒，并在间质背景下有明显的细胞间变
s68 Sentence Dramatic improvements in survival have been achieved for children and adolescents with cancer 儿童与青少年肿瘤患者的生存时间已得到大幅提高
s69 Sentence The genetics of tumors in children with hemihyperplasia have not been clearly defined 偏侧发育过度患儿的肿瘤基因学目前尚不明确
s70 Sentence Widespread hepatitis B immunization has decreased the incidence of hepatocellular carcinoma in Asia 亚洲地区的肝细胞癌发病率已在降低，这主要得益于乙肝疫苗的广泛接种
s71 Sentence Sorafenib has resulted in improved progression-free survival in adults with advanced hepatocellular carcinoma 研究显示索拉菲尼可延长晚期肝细胞癌成人患者的无进展生存时间
s72 Sentence Combination chemotherapy has been demonstrated to have significant benefit in children with hepatoblastoma 研究显示联合化疗可使肝母细胞瘤患儿有显著获益
s73 Sentence Approximately 20% to 30% of children with hepatoblastoma are stage I or II 约有20%-30%的肝母细胞瘤患儿为I期或II期
s74 Sentence If the primary tumor remains unresectable, an orthotopic liver transplantation may be performed 如果原发肿瘤仍然无法切除，可选择原位肝移植
s75 Sentence This suggests that adjuvant chemotherapy may benefit children with completely resected hepatocellular carcinoma 这一结果提示，辅助化疗对于完全切除的肝细胞癌患者可能有益
s76 Sentence Risk factors associated with hepatoblastoma and hepatocellular carcinoma are described in Table 1 与肝细胞癌与肝母细胞瘤相关的危险因素如见表1
s77 Sentence Extremely high levels of beta-hCG are associated with infantile choriocarcinoma of the liver 极高水平的β-hCG可提示婴儿肝绒毛膜癌
s78 Sentence Resection or orthotopic liver transplant will take place after four cycles of C5VD 进行4程C5VD后进行手术切除或者原位肝移植术
s79 Sentence In infants with hepatic hemangiomas or hemangioendotheliomas, which can be diagnosed by imaging 可以靠影像学诊断的婴儿肝脏血管瘤或血管内皮细胞瘤
s80 Sentence (Refer to the PDQ summary on Adult Primary Liver Cancer Treatment for more information.) （更多信息请参见PDQ总结成人原发性肝癌的治疗）
s81 Sentence Hepatocellular carcinoma is often extensively invasive or multicentric, and less than 30% are resectable 而肝细胞癌则多为弥漫性或多中心病灶，只有不到30%的情况适合切除
s82 Sentence Embryonal epithelial cells resembling the liver epithelium at 6 to 8 weeks of gestation 胚胎型上皮细胞，类似于6-8周胚胎期的肝上皮细胞
s83 Sentence Distinctive features are characteristic intracellular hyaline globules and marked anaplasia on a mesenchymal background 其特征包括细胞内透明颗粒，并在间质背景下可见明显的细胞间变
s84 Sentence Radiation therapy, even in combination with chemotherapy, has not cured children with unresectable tumors 单独放疗或放疗联合化疗无法治愈不可切除的肿瘤
s85 Sentence About 20% of tumors have stromal derivatives such as osteoid, chondroid, and rhabdoid elements 约有20%的肿瘤有间质化成分如骨样、绒毛膜样、横纹肌样成分等
s86 Sentence The age of onset of liver cancer in children is related to tumor histology 儿童肝癌的发病年龄随肝癌的病理类型不同
s87 Sentence The presurgical PRETEXT staging system may add prognostic information compared with postsurgical staging alone 基于术前的PRETEXT分期系统与仅基于术后信息分期相比对预后的提示意义更大
s88 Sentence Hepatoblastoma arises from precursors of hepatocytes and can have several morphologies, including the following: 肝母细胞瘤起源于肝细胞前体，可有多种形态，包括：
s89 Sentence Other somatic overgrowth syndromes, such as Simpson-Golabi-Behmel syndrome, may also be associated with hepatoblastoma 其他躯体过度生长综合征如Simpson-Golabi-Behmel综合征，也可能与肝母细胞瘤相关
s90 Sentence If possible, this is followed by surgical resection of primary tumor and extrahepatic disease 如果可以切除，应当对原发肿瘤和肝外病灶进行手术切除
s91 Sentence Tumor involves only one liver sector; three adjoining liver sectors are free of tumor 肿瘤局限于一个分区，三个相邻分区无肿瘤
s92 Sentence Tumor involves all four liver sectors; there is no liver sector free of tumor 肿瘤浸润四个肝分区，不存在无肿瘤分区
s93 Sentence However, use of aggressive chemotherapy regimens seems to have improved the overall survival (OS) 但高强度化疗方案似乎可以提高总生存率（OS）
s94 Sentence Thus, complete resection of a pure fetal hepatoblastoma may preclude the need for chemotherapy 因此，对单纯胎儿型肝母细胞瘤行完全切除后可以免除化疗
s95 Sentence The tumor is either unresectable or the tumor is resected with gross residual tumor 肿瘤无法切除或切除后肉眼可见残留肿瘤
s96 Sentence Aggressive treatment for this histology is under investigation in the current COG study, COG-AHEP0731 COG目前正在研究对于小细胞未分化型肝母细胞瘤的强化治疗，研究编号COG-AHEP0731
s97 Sentence Phase I and phase II clinical trials may be appropriate and should be considered 可选择参与I期或II期临床试验
s98 Sentence There are three ways in which surgery is used to treat primary pediatric liver cancer: 治疗儿童原发肝癌的手术方式包括以下三种：
s99 Sentence Patients with small cell undifferentiated hepatoblastoma whose tumors are unresectable have an especially poor prognosis 不可切除的小细胞未分化型肝母细胞瘤通常预后极差
s100 Sentence If possible, isolated metastases should be resected completely in patients whose primary tumor is controlled 在可行的情况下，一旦原发肿瘤灶得到控制，应当对孤立的转移灶进行完全手术切除
s101 Sentence In stage IV hepatoblastoma, there is distant metastasis regardless of the extent of liver involvement 不论肝脏受累程度如何，存在远处转移的肝母细胞瘤分期为IV期
s102 Sentence Chemoembolization or liver transplant should be considered for those with isolated recurrence in the liver 存在孤立性肝复发病灶的患者应当考虑进行化疗栓塞术或肝移植术
s103 Sentence Table 2. Diagnostic Differences Between Undifferentiated Embryonal Sarcoma of the Liver and Biliary Tract Rhabdomyosarcoma 表2. 肝脏未分化胚胎性肉瘤与胆道横纹肌肉瘤的鉴别诊断
s104 Sentence Many UESL contain diverse elements of mesenchymal cell maturation, such as smooth muscle and fat 许多UESL含有多重间质细胞成熟成分，例如平滑肌与脂肪
s105 Sentence The cells of hepatocellular carcinoma are epithelial while hepatoblastoma has a less differentiated embryonal appearance 肝细胞癌的细胞是上皮来源，而肝母细胞瘤则是分化程度较低的胚胎细胞状
s106 Sentence Liver transplantation has on occasion been used successfully to treat an otherwise unresectable primary tumor 肝移植偶可成功治疗无法切除的原发肿瘤
s107 Sentence Patients who underwent orthotopic liver transplant are included in all of the international study results 所有全球性研究结果中均包含了进行原位肝移植的患者
s108 Sentence Stage III tumors with an AFP level less than 100 ng/ml ( high risk ) AFP水平不足100ng/mL的III期肿瘤（高危）
s109 Sentence No particular treatment has proven effective for metastatic hepatocellular carcinoma in the pediatric age group 目前对于有转移灶的儿童肝细胞癌并无特殊的治疗方法
s110 Sentence Chemotherapy followed by reassessment of surgical resectability followed by complete surgical resection of primary tumor 化疗后重新对外科可切除性进行评估，并对原发肿瘤进行完全手术切除
s111 Sentence Surgical resection of distant disease has also contributed to the cure of children with hepatoblastoma 对于远处病灶的外科切除可提高肝母细胞瘤患儿的治愈率
s112 Sentence Chemotherapy has been much more successful in the treatment of hepatoblastoma than in hepatocellular carcinoma 化疗对肝母细胞瘤的效果远优于肝细胞癌
s113 Sentence However, living donor liver transplants are more common with children and the outcome is similar 儿童通常多接受活体肝移植，预后类似
s114 Sentence Tumor involves one or two liver sectors; two adjoining liver sectors are free of tumor 肿瘤局限于一至两个分区，两个相邻分区无肿瘤
s115 Sentence Neoadjuvant chemotherapy can be effective in decreasing an unresectable primary tumor mass, resulting in resectability 对于无法切除的原发肿瘤，新辅助化疗可以帮助缩小肿瘤，使之可切除
s116 Sentence No specific treatment has proven effective for unresectable hepatocellular carcinoma in the pediatric age group 目前对于无法切除的儿童肝细胞癌并无特殊的有效治疗方法
s117 Sentence Epithelioid hemangioendothelioma is a rare vascular cancer that occurs in the liver and other organs 上皮样血管内皮细胞瘤是一种罕见的肝脏和其他器官血管瘤
s118 Sentence It has been suggested that adjuvant chemotherapy following transplant may decrease the risk of tumor recurrence 研究认为移植术后的辅助化疗有助于降低肿瘤复发风险
s119 Sentence These tumors are usually very friable and hemorrhagic and may present with bleeding into the tumor 这类肿瘤脆性较大，易出血，可有肿瘤内出血表现
s120 Sentence The outcome for metastatic hepatoblastoma at diagnosis is poor, but long-term survival and cure is possible 诊断时已有转移的肝母细胞瘤患者预后较差，但长时间生存和治愈仍有可能
s121 Sentence The presence or absence of metastases is noted, but it does not alter the PRETEXT stage 需注意有无转移，但是并不影响PRETEXT分期
s122 Sentence The incidence of hepatoblastoma is increased 1,000-fold to 10,000-fold in infants and children with Beckwith-Wiedemann syndrome 患有Beckwith-Wiiedemann综合征的婴儿或儿童患肝母细胞瘤的风险提高了1,000至10,000倍
s123 Sentence The decision as to which surgical approach to use depends on many factors including the following: 对手术方式的选择主要基于以下因素：
s124 Sentence An alternative treatment approach might be transarterial chemoembolization followed by complete surgical resection of primary tumor 替代治疗方案包括经肝动脉化疗栓塞后对原发肿瘤行完全切除术
s125 Sentence Those who cannot be rendered free of extrahepatic disease will continue chemotherapy and not undergo transplant 无法完全清除肝外病灶的患者将继续进行化疗，不进行移植
s126 Sentence In stage III hepatoblastoma, there is no distant metastases and one of the following is true: 无远处转移灶伴至少一项以下情况的肝母细胞瘤为III期：
s127 Sentence The diagnosis can be made by imaging and findings of extremely high beta-human chorionic gonadotropin levels 可以靠影像学和β-人绒毛膜促性腺激素β（β-hCG）血清水平显著升高诊断
s128 Sentence If possible, stage IV patients with resected primary tumor should have remaining pulmonary metastases surgically removed 如可能，IV期肝母细胞瘤患者在对原发肿瘤切除术后如有残留的肺转移灶，也应行手术切除
s129 Sentence A randomized clinical trial demonstrated comparable efficacy with cisplatin/vincristine/fluorouracil and cisplatin/doxorubicin in the treatment of hepatoblastoma 一项随机临床试验认为，顺铂/长春新碱/氟尿嘧啶联合化疗和顺铂/阿霉素联合化疗治疗肝母细胞瘤的有效性相似
s130 Sentence Completely excised tumor of purely fetal and favorable histology may be carefully followed without further therapy 这些单纯胎儿型或其他良性组织学分类的肝母细胞瘤在实行完全外科切除后需要密切观察，可能无须联合其他治疗
s131 Sentence The AFP and beta-hCG tumor markers are very helpful in diagnosis and management of liver tumors 在肝脏肿瘤的诊断和治疗中，AFP和β-hCG是非常有用的指标
s132 Sentence Some UESLs arising from mesenchymal hamartomas of the liver may have complex karyotypes not involving 19q13.4 另一些起源于肝间叶错构瘤的USEL则有不累及19q13.4的复杂核型
s133 Sentence If the tumor is completely removed, two postoperative courses of the same chemotherapy should be given 若肿瘤可完全切除，术后应继续使用2程同方案化疗
s134 Sentence Standard-risk was defined as tumor confined to the liver and not involving more than three sectors 标准风险被定义为：病灶局限于肝脏，且累及不超过3个肝区
s135 Sentence Infants are often anemic and can be unstable at presentation due to hemorrhage from the tumor 患儿常出现贫血，肿瘤出血可导致体征不稳定
s136 Sentence The generally accepted approach is to resect the primary tumor mass in the liver when possible 目前公认的治疗方法是在可行的情况下尽量切除原发肿瘤
s137 Sentence Application of the Milan criteria for UNOS selection of recipients of deceased donor livers is controversial 在UNOS选择死亡死亡供体肝脏时是否应使用米兰标准仍有争议
s138 Sentence The utility of radiation therapy is questioned because the liver cannot tolerate high doses of radiation 由于肝脏无法耐受高剂量放射，因此放疗在肝肿瘤中的应用有限
s139 Sentence In rare cases, chemotherapy has eradicated pulmonary metastases and eliminated multinodular tumor foci in the liver 化疗极少根除肺转移灶或肝内多结节性肿瘤灶
s140 Sentence None of the 11 patients underwent a second resection and only one patient received radiation therapy postoperatively 这11例患者均未接受二次手术，只有1例患者接受术后放疗
s141 Sentence however, the presence of microscopic residual tumor at the surgical margin does not preclude a favorable outcome 但镜下见切缘残留肿瘤并不一定表示预后不良
s142 Sentence Half of these survivors had developed progressive disease that was successfully treated with surgery and other interventions 这些生存者中一半患者肿瘤进展，但是可通过外科或其他干预方法成功治疗
s143 Sentence Initial surgical removal of the tumor mass may be difficult because of its friability and hemorrhagic tendency 肿瘤脆性高出血倾向严重，因此对肿瘤的首次外科切除有一定困难
s144 Sentence In infantile hepatic choriocarcinoma, which can be diagnosed by imaging and markedly elevated beta-human chorionic gonadotropin (beta-hCG) 婴儿肝绒毛膜癌，可以靠影像学和β-人绒毛膜促性腺激素β（β-hCG）血清水平显著升高诊断
s145 Sentence A study of 50 sequential children with apparent sporadic hepatoblastoma reported five children (10%) had APC mutations 一项对50例散发性肝母细胞瘤患儿进行的研究中，5例患儿（10%）查出APC突变
s146 Sentence Fibrolamellar carcinoma is thought to be associated with an improved prognosis and is not associated with cirrhosis 纤维板层型肝癌预后较好，与肝硬化不相关
s147 Sentence Either mechanism can be associated with an increased incidence of embryonal tumors, including Wilms tumor and hepatoblastoma 这些机制均与Wilm瘤和肝母细胞瘤等胚胎肿瘤的发病率升高有关
s148 Sentence Strong clinical and histological evidence suggest that UESL can arise within preexisting mesenchymal hamartomas of the liver 现有的大量临床和组织学证据认为，UESL可能起源于已有的肝间叶错构瘤
s149 Sentence The tumor cells may vary in regions of the tumor between classical hepatoblastoma and obvious hepatocellular carcinoma 肿瘤部位的细胞介于典型的肝母细胞到明显的肝细胞癌细胞之间
s150 Sentence This preliminary study provides some evidence that screening children with hepatoblastoma for APC mutations may be appropriate 这项初步研究提供了一定的证据支持对肝母细胞瘤患儿行APC筛查的合理性
s151 Sentence The imaged liver is divided into four sectors and involvement of each sector with tumor is determined 肝在影像学中分为四个区域，需确定每个区域是否受累
s152 Sentence Orthotopic liver transplantation provides an additional treatment option for patients whose tumor remains unresectable after preoperative chemotherapy 原位肝移植作为另一种治疗选择，可用于术前化疗后肿瘤仍无法满足切除标准的患儿
s153 Sentence Other treatment approaches such as transarterial chemoembolization, have been used for patients with inoperable stage III hepatoblastoma 其他治疗方法如经动脉化疗栓塞术被用于无法接受手术的III期肝母细胞瘤患儿
s154 Sentence Small cell undifferentiated hepatoblastoma is an uncommon hepatoblastoma variant that represents a few percent of all hepatoblastomas 小细胞未分化型肝母细胞瘤是一种不常见的肝母细胞瘤类型，仅占所有肝母细胞瘤的百分之几
s155 Sentence Addition of doxorubicin to vincristine/fluorouracil/cisplatin is under clinical evaluation in the Children's Oncology Group (COG) study COG-AHEP0731 儿童肿瘤协作组（COG）研究COG-AHEP0731正在评估将阿霉素加入长春新碱/氟尿嘧啶/顺铂联合化疗的临床作用
s156 Sentence Hepatocellular carcinoma also differs from hepatoblastoma in that it often arises in a previously abnormal, cirrhotic liver 两者的另一个不同点则是肝细胞癌患者通常有肝脏异常、肝硬化病史
s157 Sentence Intravenous invasion, positive lymph nodes, and contiguous spread did not have a significant adverse effect on outcome 静脉侵犯、淋巴结阳性和邻近组织扩散对预后无显著不良影响
s158 Sentence A biopsy of the tumor is always indicated to secure the diagnosis of a liver tumor except: 需要对肿瘤进行活检以确诊肝癌，以下情况除外：
s159 Sentence Tumor involvement of the vena cava, hepatic veins, and portal vein, and extrahepatic extension are also noted 是否累及下腔静脉、肝静脉和门静脉、是否有肝外浸润均需记录
s160 Sentence In the presence of features predicting unresectability, early coordination with a pediatric liver transplant service is desirable 若存在提示不可切除的疾病特征，应及早联系儿童肝移植机构进行协调
s161 Sentence Transarterial chemoembolization has been used in a few children to successfully shrink tumor size to permit resection 经动脉化疗栓塞术对一些儿童患者可减小肿瘤大小，使之可切除
s162 Sentence Lack of a significant decrease of AFP levels with treatment may predict a poor response to therapy 若治疗过程中未见AFP显著下降，可能提示患儿对于此治疗的反应较差
s163 Sentence If the tumor can be completely excised by an experienced surgical team, less postoperative chemotherapy may be needed 如果有经验的外科医师团队可完全切除肿瘤，则术后化疗的需要减少
s164 Sentence Use of neoadjuvant methotrexate in infantile choriocarcinoma, although often resulting in a response, has not been uniformly successful 而虽然绒毛膜癌患儿对甲氨蝶呤新辅助化疗有反应，但并不总能治愈
s165 Sentence Most of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials 大多数儿童癌症的治愈方法就是通过这样的临床研究发现的
s166 Sentence Liver transplantation has recently been associated with significant success in the treatment of children with unresectable hepatic tumors 对于无法切除的肝癌患儿，近年来报道的肝移植的成功率很高
s167 Sentence (Refer to the Hemangioendothelioma section in the PDQ summary on Childhood Soft Tissue Sarcoma Treatment for more information.) （更多信息参见PDQ总结儿童软组织肉瘤的治疗：血管瘤部分）
s168 Sentence A regimen of intensified platinum therapy with alternating cisplatin and carboplatin was associated with a decrease in EFS 交替使用顺铂和卡铂的强化含铂类药物化疗可降低EFS
s169 Sentence The former are closely packed nests of medium-sized cells with clear cytoplasm, distinct cell margins, and vesicular nuclei 前者组织密集，主要为中等大小细胞，胞质透明，边界清楚，核呈空泡状
s170 Sentence In Europe, all children with hepatoblastoma are treated with chemotherapy prior to attempted resection of the primary tumor 在欧洲，所有肝母细胞瘤患儿在接受原发肿瘤切除术前均先化疗
s171 Sentence The standard regimen is four courses of cisplatin/vincristine/fluorouracil or doxorubicin/cisplatin combination chemotherapy followed by attempted complete tumor resection 标准化疗方案为4程顺铂/长春新碱/氟尿嘧啶或顺铂/阿霉素联合化疗，化疗后尝试进行完全肿瘤切除
s172 Sentence The AFP level can be elevated due to a benign tumor, as well as a malignant solid tumor 因为在良性、恶性实体瘤中均可见AFP升高
s173 Sentence The COG study COG-AHEP0731 (Combination Chemotherapy in Treating Children With Newly Diagnosed Hepatoblastoma) incorporates the following risk groups: COG研究COG-AHEP0731（联合化疗治疗新诊断的肝母细胞瘤患儿）使用以下危险分组：
s174 Sentence Undifferentiated embryonal sarcoma of the liver is so rare that only small series have been published regarding treatment 肝脏未分化胚胎性肉瘤极其罕见，关于该肿瘤的治疗仅有极少数小规模病例分析
s175 Sentence Historically, complete surgical resection of the primary tumor has been required to cure malignant liver tumors in children 历史上，完全切除原发肿瘤是被认为是治愈儿童恶性肝癌的方法
s176 Sentence A combination of ifosfamide, cisplatin, and doxorubicin has also been successfully used in the treatment of advanced-stage disease 异环磷酰胺、顺铂和阿霉素联合化疗被成功用于治疗晚期肝母细胞瘤
s177 Sentence A North American randomized clinical trial demonstrated comparable efficacy with cisplatin/vincristine/fluorouracil and cisplatin/doxorubicin in the treatment of hepatoblastoma 一项北美的随机临床试验发现顺铂/长春新碱/氟尿嘧啶联合化疗和顺铂/阿霉素联合化疗对肝母细胞瘤的有效性相似
s178 Sentence Metastasis from placenta to maternal tissues occurs in many cases, necessitating beta-human chorionic gonadotropin (beta-hCG) testing of the mother 很多病例发生胎盘向母体组织的转移，因此母亲应当进行人绒毛膜促性腺激素β（β-hCG）水平检验
s179 Sentence In a separate three-institution study for children with hepatocellular carcinoma, the overall 5-year disease-free survival rate was approximately 60% 另一项三中心研究显示，肝细胞癌患儿的5年总无病生存率约为60%
s180 Sentence Those whose tumor responds to VI will also receive two additional cycles of VI, totaling ten cycles of chemotherapy 对VI联合化疗反应良好的肿瘤患者再行2程VI，使总化疗达10程
s181 Sentence Although AFP is elevated in most children with hepatic malignancy, it is not pathognomonic for a malignant liver tumor 尽管多数儿童肝癌患者的AFP水平升高，但AFP并不能用于确诊肝脏恶性肿瘤
s182 Sentence Two histologic subtypes have clinical relevance: pure fetal histology throughout the tumor and foci of small cell undifferentiated cells 有临床相关性的两种组织学亚型为：全肿瘤呈单纯胚胎型，或者存在小细胞未分化灶
s183 Sentence Histologically, small cell undifferentiated hepatoblastoma is typified by a diffuse population of small cells with scant cytoplasm resembling neuroblasts 组织学上，小细胞未分化型肝母细胞瘤的特点为镜下可见弥漫分布的小细胞，胞质少，类神经母细胞
s184 Sentence Very low risk Stage I tumor with pure fetal histology and an alpha-fetoprotein (AFP) level greater than 100 ng/ml 极低危组织学为单纯胎儿型且甲胚球蛋白（AFP）水平超过100ng/mL的I期肿瘤
s185 Sentence The cisplatin/doxorubicin used in the international studies appears to be less toxic than that in the North American study 但国际研究中顺铂/阿霉素联合方案的毒性似乎低于北美研究使用的同种治疗
s186 Sentence The use of neoadjuvant chemotherapy followed by complete gross surgical resection of the primary tumor is necessary for cure 新辅助化疗加上对原发肿瘤的完全手术切除是治愈的必要方法
s187 Sentence The majority of patients were treated with chemotherapy regimens often used for pediatric rhabdomyosarcoma or Ewing sarcoma without cisplatin 大多数患者使用的化疗方案不含顺铂，和儿科横纹肌肉瘤或尤文氏肉瘤患儿的方案相同
s188 Sentence If extrahepatic disease is not resectable (post neoadjuvant chemotherapy), alternative treatment approaches may include the following: Nonstandard chemotherapy agents 如果肝外病变在新辅助化疗后仍无法切除，其它可选的治疗包括：非标准化疗
s189 Sentence The 5-year OS for PRETEXT stage 4 patients, including those with metastasis and/or extrahepatic disease, was 1 in 13 PRETEXT4期患者，包括有转移灶和（或）肝外病变的患者，其5年OS为1/13
s190 Sentence Patients with stage I tumors appear to have increased risk of treatment failure when small cell elements are present I期患者的肿瘤中若有小细胞成分存在，则治疗失败的风险较高
s191 Sentence The European PRETEXT staging system for hepatoblastoma categorizes the primary tumor based on extent of liver involvement at diagnosis 欧洲PRETEXT分期系统适用于肝母细胞瘤，按照诊断时肝脏的受累程度对原发肿瘤进行分期
s192 Sentence In a randomized trial, seven of eight patients with stage I hepatocellular carcinoma survived disease free after adjuvant cisplatin-based chemotherapy 在一项随机临床研究中，8例I期肝细胞癌患儿在含顺铂辅助化疗后有7例患者实现无病生存
s193 Sentence In several Asian countries, the incidence of hepatocellular carcinoma in children is 10 times more than that in North America 一些亚洲国家中，儿童肝细胞癌发病率比北美高10倍
s194 Sentence A regimen of intensified platinum therapy with alternating cisplatin and carboplatin was associated with a decrease in event-free survival (EFS) 交替使用顺铂和卡铂的加强含铂类化疗降低无事件生存（EFS）率
s195 Sentence It presents as an abdominal mass, often with pain or malaise, usually between the ages of 5 and 10 years 肝脏未分化胚胎性肉瘤临床表现为腹部肿块，高发年龄为5-10岁，患儿常有疼痛、乏力等不适
s196 Sentence The overall 5-year survival rate for children with hepatoblastoma is 70%, but is only 42% for those with hepatocellular carcinoma 肝母细胞瘤患儿的5年总生存率为70%，但肝细胞癌患儿的5年总生存率只有42%
s197 Sentence Clinical diagnosis may be made without biopsy based on extremely high serum beta-hCG levels and normal alpha-fetoprotein levels for age 可根据极高的β-hCG水平诊断而无需活检，其甲胎蛋白水平与年龄正常水平相当
s198 Sentence Pure fetal histology (4% of hepatoblastomas) have a 3- to 5-year OS rate of 100% with minimal or no chemotherapy 单纯胎儿型（4%的肝母细胞瘤），接受少量化疗或无化疗的情况下，3年或5年的OS均为100%
s199 Sentence Chemotherapy followed by reassessment of surgical resectability. If the primary tumor remains unresectable, an orthotopic liver transplantation may be performed 化疗后重新对外科可切除性进行评估，若仍无法切除，可进行原位肝移植
s200 Sentence The liver tumors are staged by interpretation of computerized tomography or ultrasound with or without additional imaging by magnetic resonance 肝肿瘤则通过CT、超声加或不加MRI分期
s201 Sentence Studies in adults in China suggest that repeated hepatic transarterial chemoembolization before surgery may improve the outcome of subsequent hepatectomy 中国的一项成人研究显示，肝切除术前反复进行经肝动脉化疗栓塞术可能改善后续肝切除术的预后
s202 Sentence Perhaps, screening children with hepatoblastoma for APC mutations may be appropriate, as they should be followed for potential colon cancer 因此，对有APC突变的患儿进行肝癌筛查有一定道理，对结肠癌的筛查也应一同进行
s203 Sentence Cryosurgery, intratumoral injection of alcohol, and radiofrequency ablation can successfully treat small (<5 cm) tumors in adults with cirrhotic livers 冷冻疗法、肿瘤内注射乙醇和射频消融术可能成功治疗成年肝硬化患者的小型（<5cm）肿瘤灶
s204 Sentence A review of the world experience has documented a posttransplant survival rate of 70% to 80% for children with hepatoblastomas 一项全球范围的回顾性研究发现，肝母细胞瘤患儿的移植后生存率达70%-80%
s205 Sentence Some studies have reported favorable outcomes for this group of patients, while others have noted high rates of hepatoblastoma recurrence 一些研究报道了此类患者预后较佳，也有一些研究显示术后肝母细胞瘤复发率较高
s206 Sentence The European PRETEXT staging system can also be used to restage patients after surgery, which has been termed POSTTEXT staging 欧洲PRETEXT分期系统也可以用于对术后患者重新分期，此时称为POSTTEXT分期
s207 Sentence If extrahepatic disease is in complete remission and the primary tumor remains unresectable, an orthotopic liver transplantation may be performed 若肝外病变已完全缓解，但原发肿瘤仍无法切除，可进行原位肝移植
s208 Sentence Stage increases and prognosis decreases as the number of liver sectors radiologically involved with tumor increases from one to four 影像学中随着肿瘤累及区域从一个增加至四个，分期也随之增高，预后变差
s209 Sentence Ten of 17 patients survived in their first complete remission, and one patient survived in his or her third complete remission 17例患儿中10例在首次完全缓解后存活，1例患儿在第三次完全缓解后存活
s210 Sentence Occasional patients may benefit from treatment with cisplatin/doxorubicin therapy, especially if localized hepatic tumor shrinks adequately to allow resection of disease 少数患者经顺铂/阿霉素联合化疗后改善预后，尤见于化疗缩小肿瘤后可行手术切除时
s211 Sentence When possible, resection of areas of locally invasive disease, such as in the diaphragm, and of isolated brain metastasis is recommended 可行的情况下，建议切除局灶性侵犯灶，如膈肌或孤立性脑转移灶
s212 Sentence Low risk Stage I and II tumors and no small cell undifferentiated elements and an AFP level greater than 100 ng/ml 低危无小细胞未分化成分且AFP水平超过100ng/mL的I期与II期肿瘤
s213 Sentence In an international study, pre-resection neoadjuvant chemotherapy (doxorubicin and cisplatin) was given to all children with hepatoblastoma with or without metastases 一项国际性研究对所有肝母细胞瘤患儿（不论存在转移灶与否）均行切除术前的新辅助化疗（阿霉素联合顺铂）
s214 Sentence The responders had a superior tumor resectability and survival, although the OS was 28% and only those undergoing complete resection survived 尽管缓解者总生存率（OS）仅为28%，但他们的肿瘤可切除性与生存率提高，只有接受完全手术切除的患者生存
s215 Sentence Fortunately, cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975 尽管自1975年起，儿科肿瘤的发生率缓慢上升，但令人欣慰的是，儿童与青少年肿瘤的发生总体较为罕见
s216 Sentence Beckwith-Wiedemann syndrome can be caused by genetic mutations and be familial, or much more commonly, by epigenetic changes and be sporadic Beckwith-Wiiedemann综合征可由基因突变引起，或具有家族遗传性，而更常见的则为表观遗传变异引起，或为散发
s217 Sentence Chemotherapy followed by reassessment of surgical resectability. If the primary tumor remains unresectable, alternative treatment approaches may include the following: Cryosurgery 化疗后重新对外科可切除性进行评估， 若仍无法切除，可考虑使用以下替代疗法：冷冻疗法
s218 Sentence Their retrospective PRETEXT stages were stage I (n = 4), stage II (n = 6), and stage III (n = 2) 这些患儿的回顾性PRETEXT分期为I期（n=4），II期（n=6）与III期（n=2）
s219 Sentence In hepatocellular carcinoma, vascular invasion, distant metastases, lymph node involvement, tumor size, and male gender were significant risk factors for recurrence 肝细胞癌复发的主要危险因素包括：血管侵犯、远处转移、淋巴结受累、肿瘤大小和男性
s220 Sentence The rates of resection were similar for the cisplatin (95%) and cisplatin/doxorubicin (93%) groups, as were OS (95% and 93%), respectively 两组的切除率相似，顺铂组为95%，顺铂联合阿霉素组为93%，其OS亦类似（分别为95%和93%）
s221 Sentence The half-life of AFP is 5 to 7 days, and by age 1 year, it should be less than 10 ng/ml AFP的半衰期为5-7天，1周岁时时小儿AFP水平应当低于10ng/mL
s222 Sentence Beta-hCG levels may also be elevated in children with hepatoblastoma or hepatocellular carcinoma, which may result in isosexual precocity in boys 肝母细胞瘤或者肝细胞癌患儿也可能有β-hCG水平升高，可造成男童的同性性早熟
s223 Sentence A small focus of undifferentiated small cell histology within an otherwise pure fetal histology tumor must be treated with aggressive chemotherapy 但当此类单纯胎儿型肿瘤中出现未分化小细胞型病灶时，必须进行积极化疗
s224 Sentence In 74 patients with PRETEXT stage 4 tumors, 22 of whom also had metastases, 31 became resectable and 26 underwent transplant 74例PRETEXT4期患儿中，22例有转移灶，31例肿瘤可切除，26例行肝移植术
s225 Sentence Hepatoblastoma of pure fetal histology: For tumors of pure fetal histology, complete surgical resection followed by watchful waiting or single-agent doxorubicin 单纯胎儿型肝母细胞瘤：对单纯胎儿型肿瘤行完全外科手术切除，术后予以密切观察或单独使用阿霉素
s226 Sentence (Refer to the PDQ Supportive and Palliative Care summaries for specific information about supportive care for children and adolescents with cancer.) （关于儿童与青少年癌症支持治疗的详细信息，参见PDQ支持与姑息治疗总结）
s227 Sentence Lack of detection of INI1 by immunohistochemistry is another characteristic shared by some small cell undifferentiated hepatoblastomas and malignant rhabdoid tumors 小细胞未分化型肝母细胞瘤和恶性横纹肌瘤的另一个相似点是，免疫组化结果显示两者均无INI1缺失
s228 Sentence This suggested that patients with pure fetal histology tumors might not need chemotherapy after complete resection of a stage I tumor 这意味着分期为I期的单纯胎儿型肝母细胞瘤在完全切除后可无需化疗
s229 Sentence Several specific types of nonviral liver injury and cirrhosis are associated with hepatocellular carcinoma in children, including tyrosinemia and biliary cirrhosis 一些特殊的非病毒性肝损伤也与儿童肝细胞癌相关，包括酪氨酸血症和胆汁性肝硬化
s230 Sentence Tyrosinemia patients should be screened for hepatoblastoma on a regular basis, whether or not they are treated with 2-(2 nitro-4-3 trifluoro-methylbenzoyl)-1, 3-cyclohexanedione 不论是否接受2-（2硝基-4-三氟甲基苯甲酰基）-1,3-环己二酮治疗，酪氨酸血症患儿均应定期进行肝母细胞瘤筛查
s231 Sentence The COG is investigating the use of PRETEXT/POSTTEXT stage before and after chemotherapy to determine the optimal surgical approach ( COG-AHEP0731 ) COG目前正在研究在化疗前后使用PRETEXT或POSTTEXT分期来评估最佳手术方案（COG-AHEP0731）
s232 Sentence If any small cell undifferentiated elements are present in stage I or II hepatoblastoma, the 3-year survival rate is 40% to 70% 对于任何有小细胞未分化成分的I期或II期肝母细胞瘤，3年生存率为40%-70%
s233 Sentence In another study, platinum- and doxorubicin-based multidrug chemotherapy induced complete regression in approximately 50% of patients, with subsequent 3-year EFS of 56% 另一项研究中，含铂类与阿霉素的多药联合化疗使约50%患儿完全缓解，其后续的3年EFS达到了56%
s234 Sentence This strategy resulted in an overall survival (OS) of 75% at 5 years after diagnosis for all children entered in the study 该方法使得参与研究的患儿诊断后5年总生存率（OS）达到75%
s235 Sentence The 3-year OS of this group was 69% ± 11%. The 3-year OS of all patients with metastases was 62% ± 12% 此组患者的3年OS为69%±11%，而所有有转移灶患者的3年OS为62%±12%
s236 Sentence Data to date cannot rule out the possibility that predisposition to hepatoblastoma may be limited to a specific subset of APC mutations 目前的证据不能排除对肝母细胞瘤的易感性可能仅限于某些APC突变类型
s237 Sentence The level of AFP at diagnosis and rate of decrease in AFP during treatment should be compared with the age-adjusted normal range 诊断时的AFP水平和治疗过程中AFP的下降程度应当和年龄校正后的正常水平进行对比
s238 Sentence Liver cancer is a rare malignancy in children and adolescents and is divided into two major histologic subgroups: hepatoblastoma and hepatocellular carcinoma 肝癌在儿童和青少年中罕见，主要根据组织学分为两类：肝母细胞瘤与肝细胞癌
s239 Sentence All children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia should be screened regularly by ultrasound to detect abdominal malignancies at an early stage 所有Beckwith-Wiiedemann综合征或单纯偏侧发育过度患儿应当进行定期超声检查，以早期发现腹腔恶性肿瘤
s240 Sentence A distinctive histologic variant of hepatocellular carcinoma, termed fibrolamellar carcinoma, has been described in the livers of older children and young adults 纤维板层型肝癌是一种特殊组织学类型的肝细胞癌，常见于年龄较大的儿童和青年
s241 Sentence Childhood and adolescent cancer survivors require close follow-up since cancer therapy side effects may persist or develop months or years after treatment 由于癌症治疗的副作用可能持续几个月甚至多年，可影响患儿身体生长，故需这些仍生存的儿童与青少年肿瘤患者进行密切随访
s242 Sentence Following chemotherapy, and excluding those who received liver transplant (less than 5% of patients), complete resection was obtained in 87% of children 化疗后，除接受肝移植的患儿之外（少于患者总数的5%），87%的患儿可实现完全切除
s243 Sentence Intensive platinum- and doxorubicin-based multidrug chemotherapy can induce complete regressions in approximately 50% of patients, with subsequent 3-year event-free survival of 56% 含铂类、阿霉素的强化多药化疗可以使约50%的患者实现完全切除，其后续的3年无事件生存率为56%
s244 Sentence Cisplatin-based chemotherapy has resulted in a survival rate of greater than 90% for children with postsurgical stage I and stage II disease 术后分期为I期或II期患儿行含顺铂化疗后，生存率超过90%
s245 Sentence Many mesenchymal hamartomas of the liver have a characteristic translocation with a breakpoint at 19q13.4 and several UESLs have the same translocation 许多肝间叶错构瘤有特征性染色体易位、19q13.4断裂，一些UESL亦可见相同易位
s246 Sentence Maternal gestational trophoblastic tumors are exquisitely sensitive to methotrexate, and many women, including those with distant metastases, are cured with single-agent chemotherapy 母体妊娠滋养细胞瘤对甲氨蝶呤非常敏感，大多数女性患者，包括有远处转移的患者，均可使用单药化疗治愈
s247 Sentence Treatment with cisplatin and doxorubicin may be recommended as adjuvant therapy since these are active agents in the treatment of hepatocellular carcinoma 推荐的辅助化疗药物为顺铂和阿霉素，这两种药物对肝细胞癌的作用明显
s248 Sentence Stage I and II with an AFP level greater than 100 ng/ml, non-small cell undifferentiated (low risk): Cisplatin/5-fluorouracil/vincristine for two 3-week cycles AFP水平大于100ng/mL、无小细胞未分化成分的I期与II期肿瘤（低风险）：2程顺铂/5-氟尿嘧啶/长春新碱联合化疗，每疗程为3周
s249 Sentence Because of the relative rarity of cancer in children, all children with liver cancer should be considered for entry into a clinical trial 由于儿科肿瘤相对较为罕见，肝癌的患儿均应考虑参与临床试验
s250 Sentence These abnormalities include translocations involving a breakpoint on chromosome 22q11 and homozygous deletion at the chromosome 22q12 region that harbors the SMARCB1/INI1 gene 这些异常包括染色体22q11断裂和携带SMARCB1/INI1基因的22q12染色体区域纯合缺失等
s251 Sentence Treatment planning by a multidisciplinary team of cancer specialists with experience treating tumors of childhood is required to determine and implement optimum treatment 为选择与实施最佳的治疗方案，儿科肿瘤的治疗计划应当由多科经验丰富的肿瘤专家共同制定
s252 Sentence The second international study compared 3-year OS among hepatoblastoma patients by PRETEXT stage absent of extrahepatic disease. The 3-year OS was as follows: 第二项肝母细胞瘤国际研究的受试者为无肝外病变的肝母细胞瘤患者，根据PRETEXT分期，其三年OS分别为：
s253 Sentence In two prospective trials, cisplatin plus either vincristine/fluorouracil or continuous infusion doxorubicin was ineffective in adequately treating 25 patients with metastatic hepatocellular carcinoma 2项前瞻性临床研究发现，顺铂联合长春新碱/氟尿嘧啶或顺铂联合连续输注阿霉素对得到适当治疗的25例有转移灶的肝细胞癌患者无效
s254 Sentence All of these studies are limited by small patient numbers; further study is needed to better define outcomes for this subset of patients 但鉴于所有这些研究的患者样本量均较小，仍需进一步展开研究以证实这一群患者的预后
s255 Sentence While complete surgical resection is important for all liver tumors, this is especially true for hepatocellular carcinoma because no effective chemotherapy is available 完全手术切除对所有肝脏肿瘤都非常重要，对缺乏针对性化疗的肝细胞癌的尤有意义
s256 Sentence A staging system based on operative findings and surgical resectability has been used in the United States to group children with liver cancer 在美国已经对儿童肝癌进行了基于术中发现和可切除性的分期系统
s257 Sentence All of the patients were treated with four courses of cisplatin and doxorubicin prior to surgery and received two courses of postoperative chemotherapy 所有患者均在术前接受了4程顺铂联合阿霉素化疗和2程术后化疗
s258 Sentence Undifferentiated embryonal sarcoma of the liver is a distinct clinical and pathologic entity and accounts for 2% to 15% of pediatric hepatic malignancies 肝脏未分化胚胎性肉瘤有独特的临床和病理特点，占儿童肝脏恶性肿瘤的2%-15%
s259 Sentence The responders had a superior tumor resectability and survival, although the overall survival (OS) was 28% and only those undergoing complete resection survived 尽管缓解者总生存率（OS）仅为28%，但他们的肿瘤可切除性与生存率提高，只有接受完全手术切除的患者生存
s260 Sentence Ninety percent of patients with hepatoblastoma and two-thirds of patients with hepatocellular carcinoma have a serum tumor marker, AFP, which parallels disease activity 90%的肝母细胞瘤患者和三分之二的肝细胞癌患者血清标志物AFP升高，其水平和疾病活动度平行
s261 Sentence Liver transplantation has been successful therapy for children with unresectable hepatocellular carcinoma; survival is about 60% with most deaths resulting from tumor recurrence 肝移植术对于无法切除的肝细胞癌患儿较为成功，生存率约达60%，多数患儿死亡的原因为肿瘤复发
s262 Sentence While vascular involvement was initially thought to be a contraindication to resection, experienced liver surgeons are able to perform aggressive approaches avoiding transplantation 虽然血管浸润曾被认为是手术切除的禁忌证，但经验丰富的肝外科医师仍可行积极手术，以避免移植
s263 Sentence The 3- to 5-year OS rate for children with stage IV hepatoblastoma vary widely based on published reports, from 20% to approximately 60% 不同研究报道的这类患者的3年至5年OS率差异较大，为20%-约60%不等
s264 Sentence In a study employing a well-tolerated regimen of doxorubicin/cisplatin chemotherapy, about 50% of patients with metastases at presentation survived 5 years from diagnosis 一项研究使用了耐受性较佳的阿霉素/顺铂联合化疗，约有50%发病时已有转移的患者在诊断后生存时间超过5年
s265 Sentence When sporadic, the types of embryonal tumors associated with Beckwith-Wiedemann syndrome have frequently also undergone somatic changes in the Beckwith-Wiedemann syndrome locus and IGF-2 散发病例中，与Beckwith-Wiiedemann综合征相关的胚胎肿瘤亦常有Beckwith-Wiiedemann综合征位点与IGF-2基因位点的体细胞突变
s266 Sentence Hepatitis C infection is associated with development of cirrhosis and hepatocellular carcinoma that takes decades to develop and is generally not seen in children 丙肝病毒感染和肝硬化、肝细胞癌相关，发展至肝细胞癌需数十年时间，在儿童中罕见
s267 Sentence Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard 面向儿童与青少年的临床研究大多设计为比较已有的标准治疗方案与潜在的更佳方案
s268 Sentence The goal of this study is to determine the objective response rate of sorafenib in children with refractory or relapsed tumors as specified above 此研究目的是评估索拉菲尼对上述难治性或复发性肿瘤患儿的客观缓解率
s269 Sentence Liver transplantation has been a successful therapy for children with unresectable hepatocellular carcinoma; survival is about 60% with most deaths resulting from tumor recurrence 肝移植术对于无法切除的肝细胞癌患儿较为成功，生存率约达60%，多数患儿死亡的原因为肿瘤复发
s270 Sentence The International Society of Pediatric Oncology Epithelial Liver Tumor Group (SIOPEL) compared cisplatin alone with cisplatin and doxorubicin in patients with preoperative standard-risk hepatoblastoma 国际儿童肝肿瘤协作组（SIOPEL）比较了单独应用顺铂和顺铂联合阿霉素化疗对标准风险肝母细胞瘤患儿的疗效
s271 Sentence Hepatoblastoma that would otherwise be considered very low or low risk is upgraded to intermediate risk if any small cell undifferentiated elements are found 任何原本的极低危或低危肝母细胞瘤一旦发现有小细胞未分化组织学成分，都应当立刻被分为中危
s272 Sentence For adult patients who received sorafenib, the median survival and time to radiologic progression were about 3 months longer than those who received a placebo 与安慰剂组相比，服用索拉菲尼的患者从治疗至发现影像学进展的生存时间中位数延长3个月
s273 Sentence The study also prospectively analyzed OS in patients by the presence of intraabdominal extrahepatic disease without distant metastasis (OS, 58%) and distant metastases (OS, 44%) 此研究亦前瞻性地分析了有腹腔内肝外病变但无远处转移的患者（OS：58%）和有远处转移的患者（OS：44%）的OS
s274 Sentence Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common liver malignancy in children and adolescents, comprising 9% to 13% of liver tumors 肝脏未分化胚胎性肉瘤（UESL）位列儿童与青少年常见肝脏恶性肿瘤的第三位，占肝肿瘤的9%-13%
s275 Sentence Tumor with POSTTEXT 2 with greater than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, or the portal bifurcation 影像学显示肿瘤边缘距肝中静脉、肝后下腔静脉或门静脉分支超过1cm的PRETEXT 2期肿瘤
s276 Sentence Hepatoblastomas usually occur before the age of 3 years, and approximately 90% of malignant liver tumors in children aged 4 years and younger are hepatoblastomas 肝母细胞瘤常在3岁前发病，4岁以下儿童中约90%的恶性肝癌为肝母细胞瘤
s277 Sentence COG is investigating the use of PRETEXT stage at diagnosis and after chemotherapy to determine the optimal surgical approach and its timing ( COG-AHEP0731 ) COG正在评估诊断时与化疗后的PRETEXT分期对优化手术方案及其时间的意义（COG-AHEP0731）
s278 Sentence If a hepatoblastoma is completely removed, the majority of patients survive, but less than one-third of patients have lesions amenable to complete resection at diagnosis 如果肝母细胞瘤可被完全切除，大部分患儿可存活，但只有不到三分之一的患儿在诊断时病变可完全切除
s279 Sentence The gene dosage and ensuing increased expression of insulin-like growth factor 2 (IGF-2) has been implicated in the macrosomia and embryonal tumors in Beckwith-Wiedemann syndrome 基因剂量与后续高表达的胰岛素样生长因子2（IGF-2）被认为与Beckwith-Wiiedemann综合征中巨大儿和胚胎肿瘤的发生相关
s280 Sentence A review of patients treated on a U.S. Intergroup trial suggested that resection may be done at the time of resection of the primary tumor 一项美国组间试验进行了病例回顾，研究发现切除原发肿瘤时可以同事切除转移灶
s281 Sentence Both staging systems are used in the United States, although initial resection of PRETEXT 1 and 2 hepatoblastomas are routinely undertaken in the United States 美国同时使用这两套分期系统，尽管在美国PRETEXT1和2期肝母细胞瘤通常直接进行常规切除
s282 Sentence Screening using alpha-fetoprotein (AFP) levels, in addition to abdominal ultrasound, has helped in the early detection of hepatoblastoma in children with Beckwith-Wiedemann syndrome or hemihyperplasia 甲胎蛋白（AFP）筛查也可帮助这些患儿早期发现肝母细胞瘤的存在
s283 Sentence Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics 美国儿科学会起草了关于儿童癌症治疗中心的指南，并阐明了儿童癌症治疗中心在儿童肿瘤患者治疗中的角色
s284 Sentence Hepatocellular carcinoma may also arise in very young children with mutations in the bile salt export pump ABCB11 , which causes progressive familial hepatic cholestasis 有胆盐出胞转运泵ABCB11基因突变的幼儿也可能发生肝细胞癌
s285 Sentence Non–pure fetal histology, non–small cell undifferentiated stage I and II hepatoblastomas have a 3- to 4-year OS rate of 90% to 100% with adjuvant chemotherapy 非单纯胎儿型、非小细胞未分化型的I期或II期肝母细胞瘤在接受辅助化疗的情况下，其3年至4年的OS为90%-100%
s286 Sentence The prognosis for a patient with recurrent or progressive hepatoblastoma depends on many factors, including the site of recurrence, prior treatment, and individual patient considerations 复发性或进展性肝母细胞瘤的预后取决于很多因素，包括复发部位、治疗史与患者的个人因素
s287 Sentence Mutations in the met/hepatocyte growth factor receptor gene occur in childhood hepatocellular carcinoma, and this could be one mechanism that results in a shortened incubation period 儿童肝细胞癌有Met/肝细胞生长因子受体基因的突变，这一机制可能导致了潜伏期的缩短
s288 Sentence It tends to occur at a younger age (6–10 months) compared with other cases of hepatoblastoma and is associated with AFP normal for age at presentation 与其他肝母细胞瘤相比，其发病年龄常较小（6-10个月），AFP水平为正常同龄水平
s289 Sentence Hepatocellular carcinoma is associated with hepatitis B and hepatitis C infection in adults, while in children there is an association with perinatally acquired hepatitis B virus 成人的肝细胞癌与乙肝和丙肝病毒感染密切相关，而儿童的肝细胞癌则和围产期感染乙肝病毒相关
s290 Sentence In a report of 11 cases of UESL, five arose in association with mesenchymal hamartomas of the liver, and transition zones between the histologies were noted 一篇文章报道了11例USEL，其中5例与肝间叶错构瘤相关，病理可见移行区
s291 Sentence Stage I pure fetal histology with an alpha-fetoprotein (AFP) level greater than 100 ng/ml, non-small cell undifferentiated (very low risk): Resection and observation with no chemotherapy 甲胎蛋白（AFP）水平超过100ng/mL、无小细胞未分化成分的I期单纯胎儿型肿瘤（极低风险）：切除并观察，不行化疗
s292 Sentence Many of the improvements in survival in childhood cancer have been made using new therapies that have attempted to improve on the best available, accepted therapy 为改善现有的最佳疗法，新治疗方案的诞生帮助提高了肿瘤患儿的生存率
s293 Sentence Undifferentiated sarcomas and small cell undifferentiated hepatoblastomas should be examined for loss of INI1 expression by immunohistochemistry to help rule out rhabdoid tumor of the liver 对未分化肉瘤和未分化性小细胞型肝母细胞瘤应行免疫组化检查，筛查是否缺失INI1表达，以排除肝脏横纹肌瘤
s294 Sentence In a retrospective comparison of the two staging systems at diagnosis using data from patients entered on a North American randomized trial, both staging systems predicted outcome 一项回顾性研究对比了两套分期系统对诊断的意义，此研究使用一项北美随机临床试验的患者数据，发现两个分期系统均对预后有提示意义
s295 Sentence Use of the PRETEXT staging system after chemotherapy to determine resectability (termed POSTTEXT): Tumors considered resectable after two or four cycles of chemotherapy: Tumors with POSTTEXT 1 化疗后使用PRETEXT分期系统判断肿瘤的可切除性（又称POSTTEXT）：2-4程化疗后可被切除的肿瘤有：POSTTEXT 1期肿瘤
s296 Sentence Further study will be needed to determine whether presurgical chemotherapy is preferable to resection followed by chemotherapy for children with PRETEXT stage 2, 3, and 4 hepatoblastoma 而对于PRETEXT2、3和4期肝母细胞瘤患儿，需进一步研究以评估术前化疗和术后化疗的优点
s297 Sentence There is an association between hepatoblastoma and familial adenomatous polyposis (FAP); children in families that carry the APC gene are at an 800-fold increased risk for hepatoblastoma 家族性腺瘤性息肉病（FAP）与肝母细胞瘤之间存在关联；携带APC基因的家族中儿童患肝母细胞瘤的风险升高800倍
s298 Sentence Biopsy only of PRETEXT 2 tumors with less than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, and the portal bifurcation 对影像学显示肿瘤边缘距肝中静脉、肝后下腔静脉或门静脉分支不足1cm的PRETEXT 2期肿瘤行活检
s299 Sentence In approximately 75% of children and adolescents with initially unresectable hepatoblastoma, tumors can be rendered resectable with cisplatin-based preoperative chemotherapy, and 60% to 65% will survive disease-free 肝母细胞瘤在初诊时无法切除的儿童与青少年中，约有75%的患儿在使用含顺铂术前化疗后肿瘤可切除，最终有60%-65%的患儿可达到无病生存
s300 Sentence In a study of patients with hepatoblastoma and pure fetal histology tumors, there was a 100% survival rate for patients who received four doses of single-agent doxorubicin 一项对单纯胎儿型肝母细胞瘤患儿的研究显示，接受4剂量阿霉素单药治疗的患儿生存率为100%
s301 Sentence This study showed that second resection of positive margins and/or radiation therapy may not be necessary in patients with incompletely resected hepatoblastoma whose residual tumor is microscopic 此研究显示，肝母细胞瘤切除术后有显微镜下残留病灶时，可能无须对阳性切缘进行二次手术和（或）放疗
s302 Sentence Clinical diagnosis may be made without biopsy based on tumor imaging of the liver associated with extremely high serum beta-hCG levels and normal AFP levels for age 临床诊断可以不依赖活检，而根据肝脏影像学加极高的血清β-hCG和正常的年龄校正AFP水平进行诊断
s303 Sentence Approximately 50% to 70% of children with hepatoblastoma are stage III. The 3- to 5-year OS rate for children with stage III hepatoblastoma is less than 70% 约有50%-70%的肝母细胞瘤患儿为III期，其3年至5年OS不到70%
s304 Sentence Resection of pulmonary metastases is recommended when the number of metastases is limited and is often performed at the same time as resection of the primary tumor 当肺转移灶较少时，建议进行肺转移灶切除数，转移灶切除术通常和原发肿瘤同时进行
s305 Sentence The protocol ( COG-P9645 ) did not treat children with stage I tumors of purely fetal histology with preoperative or postoperative chemotherapy unless they developed progressive disease 这一方案（COG-P9645）对I期的单纯胎儿性肿瘤患儿不建议行术前或术后化疗，除非患者肿瘤进展
s306 Sentence Hepatoblastoma with non–pure fetal histology: Gross surgical excision followed by four courses of combination chemotherapy with cisplatin, vincristine, and fluorouracil or cisplatin and doxorubicin or cisplatin alone 非单纯胎儿型肝母细胞瘤：对肿瘤进行完全切除后，行4程联合化疗，方案包括顺铂、长春新碱联合氟尿嘧啶、顺铂联合阿霉素或单独使用顺铂
s307 Sentence All 16 patients entered on this study were alive with no evidence of disease at a median follow-up of 4.9 years (range, 9 months to 9.2 years) 在中位数为4.9年（范围：9个月-9.2年）的随访期中，16例患儿均存活，且随访期末未发现疾病证据
s308 Sentence (Refer to Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.) （儿童与青少年癌症幸存患者后期作用的发生率、类型与监测请参见儿童肿瘤治疗的后期效果）
s309 Sentence Accomplishing a complete resection is imperative because rescue transplant of incompletely resected patients has an inferior outcome compared with patients who are transplanted as the primary surgical therapy 成功的完全切除对患儿及其重要，相较于以移植为首要外科治疗方案的患儿，在切除术无效后进行补救性移植的患儿预后较差
s310 Sentence Tumor involves three liver sectors and one liver sector is free of tumor or tumor involves two liver sectors and two nonadjoining liver sectors are free of tumor 肿瘤位于三个分区，另一分区无肿瘤；或肿瘤位于其中两个分区，另外两个非相邻分区无肿瘤
s311 Sentence For this reason, surgeons with experience in pediatric liver resection and transplantation should be involved early in the decision-making process for determining optimal timing and extent of resection 因此，在决定最优时机和切除范围时应及早让有丰富的儿童肝切除和肝移植术经验的儿科医师参与
s312 Sentence Although outcome was nominally higher for children receiving cisplatin/doxorubicin, this difference was not statistically significant, and the combination of cisplatin/vincristine/fluorouracil was less toxic than the regimen of cisplatin/doxorubicin 尽管研究发现使用顺铂/阿霉素联合化疗患儿的预后更好，但其差异不具统计学显著性，且顺铂/长春新碱/氟尿嘧啶联合化疗的毒性低于顺铂/阿霉素联合化疗方案
s313 Sentence The International Society of Pediatric Oncology Epithelial Liver Tumor Group (SIOPEL) uses a presurgical-based (PRETEXT) staging system, while the Children's Oncology Group (COG) uses a postsurgical-based staging system 国际儿童肿瘤协会肝上皮肿瘤组（SIOPEL）以手术前情况（PRETEXT）为分期原则，而儿童肿瘤协作组（COG）则以术后情况进行分期
s314 Sentence Disease evaluation, usually after four cycles of chemotherapy, was followed by second-look surgery when appropriate to try to remove residual primary tumor followed by additional and/or adjuvant chemotherapy 通常在4程化疗后对疾病进行评估，若条件允许，则在化疗后对原发肿瘤残留灶尝试行二次手术加（或）辅助化疗
s315 Sentence Second resection of positive margins and/or radiation therapy may not be necessary in patients with incompletely resected hepatoblastoma whose residual tumor is microscopic and who receive subsequent chemotherapy 对于没有完全切除、留有显微镜下残留病灶，且接受术后化疗的肝母细胞瘤患者，可能并不一定需要对阳性切缘行二次切除和（或）放疗
s316 Sentence There may be a role for radiation therapy in the management of incompletely resected hepatoblastoma, although a study of 154 patients with hepatoblastoma did not confirm this finding 放疗可能对无法完全切除的肝母细胞瘤有一定 效果，但一项招募了154例肝母细胞瘤患儿的研究结果与此结论并不一致
s317 Sentence In contrast, an American Intergroup protocol for treatment of children with hepatoblastoma encouraged resection at the time of diagnosis for all tumors amenable to resection without undue risk 一项美国组间治疗方案建议鼓励诊断无不合理风险的可切除肝母细胞瘤患儿立刻接受手术切除
s318 Sentence Compared with adults, the incubation period from hepatitis virus infection to the genesis of hepatocellular carcinoma is extremely short in a small subset of children with perinatally acquired virus 与成年人相比，围产期感染乙肝病毒的儿童中，有一部分感染后很快发生肝细胞癌
s319 Sentence Despite improvements in surgical techniques, chemotherapy delivery, and patient supportive care in the past 20 years, clinical trials of cancer chemotherapy for hepatocellular carcinoma have not shown improved outcome 尽管手术技术、化疗药物给药方法和患者的支持性治疗在过去的20年中得到了长足进步，但临床肝细胞癌化疗的临床试验并未发现治疗结果在改善
s320 Sentence The OS of these children appears to be substantially better than 50% when combining reports, although all series are small and most may be selected to report successful treatment 尽管文献中分析的病例数较少，又倾向于报道成功的治疗案例，但联合所有文献，这类患儿的OS远远超过50%
s321 Sentence Analysis of survival after recurrence demonstrated that some patients treated with cisplatin/vincristine/fluorouracil could be salvaged with doxorubicin-containing regimens, but patients treated with doxorubicin/cisplatin could not be salvaged with vincristine/fluorouracil 复发后生存率分析显示，曾经使用顺铂/长春新碱/氟尿嘧啶进行联合化疗的部分患者可换用含阿霉素的化疗方案，但是曾使用阿霉素/顺铂联合化疗的患者不可换用长春新碱/氟尿嘧啶方案
s322 Sentence It has been suggested that some UESLs arise from mesenchymal hamartomas of the liver, which are large benign multicystic masses that present in the first 2 years of life 有人提出UESL来源于肝间叶错构瘤，一种在2周岁前出现的较大良性多囊性肝脏包块
s323 Sentence The chromosomal abnormalities reported for small cell undifferentiated hepatoblastoma are distinct from those occurring in other hepatoblastoma subtypes and are more similar to those observed in malignant rhabdoid tumors 小细胞未分化型肝母细胞瘤的染色体异常与其他类型肝母细胞瘤的染色体异常不同，更类似于恶性横纹肌瘤的染色体异常
s324 Sentence Choriocarcinoma of the liver is a very rare tumor that appears to originate in the placenta and presents with a liver mass in the first few months of life 肝绒毛膜癌是一种起源于胎盘的罕见肿瘤，常见于几个月大的婴儿，表现为肝脏肿块
s325 Sentence The combination of cisplatin, etoposide, and bleomycin, as used in other pediatric germ cell tumors, has been effective in some patients and is followed by resection of residual mass 和其他儿童生殖细胞肿瘤类似，使用顺铂、依托泊苷和博来霉素联合化疗后对残留肿瘤进行切除
s326 Sentence Chemotherapy followed by radiation therapy followed by surgical re-exploration for patients in whom extrahepatic disease is controlled, but the primary tumor remains unresectable following treatment with standard chemotherapy regimens 对肝外病变已得到控制，但标准化疗后原发肿瘤仍无法切除的患者，放疗后可进行化疗和后续的手术探查
s327 Sentence Stage I and II with an AFP level greater than 100 ng/ml with small cell undifferentiated elements (intermediate risk): Cisplatin/5-fluorouracil/vincristine/doxorubicin for six cycles, with dexrazoxane the last two cycles P水平大于100ng/mL、存在小细胞未分化成分的I期与II期肿瘤（直接风险）：6程顺铂/5-氟尿嘧啶/长春新碱/阿霉素联合治疗，最后2程加用右雷佐生
s328 Sentence Another study of children with hepatoblastoma found a predominance of the mutation in the 5' region of the gene, but some patients had mutations closer to the 3' region 另一项对肝母细胞瘤患儿进行的研究显示，大多数突变位于基因的5’区，但也有一些患儿的突变位于3’区
s329 Sentence Experienced radiologist review is important because it may be difficult to discriminate between real invasion beyond the anatomic border of a given sector and displacement of the anatomic border 肿瘤浸润超过分区的解剖学界限与解剖学界限异位难以区分，因此需要有经验的放射科医生审核
s330 Sentence In another European study of high-risk hepatoblastoma, 11 patients had microscopic residual tumor remaining after initial surgery and received two to four postoperative cycles of chemotherapy with no additional surgery 另一项欧洲高危肝母细胞瘤研究中，11例患者初次手术后有显微镜下残留病灶，这些患者接受了2-4程术后化疗，未进行二次手术
s331 Sentence In the Children's Oncology Group (COG) study COG-P9645 , stage I pure fetal histology hepatoblastomas with two or fewer mitoses per 10 high power fields were not treated with chemotherapy 儿童肿瘤协作组（COG）研究COG-P9645显示，I期单纯胎儿型肝母细胞瘤若在每10个高倍镜视野下有少于等于两个分裂细胞，则无须使用化疗
s332 Sentence Although outcome was nominally higher for children receiving cisplatin/doxorubicin, this difference was not statistically significant, and the combination of cisplatin/vincristine/fluorouracil was significantly less toxic than the doses of cisplatin/doxorubicin used 尽管研究发现使用顺铂/阿霉素联合化疗患儿的预后更好，但其差异不具统计学差异，且顺铂/长春新碱/氟尿嘧啶联合化疗的毒性较另一种化疗方案低
s333 Sentence Early involvement with an experienced pediatric liver surgeon is especially important in patients with PRETEXT stage 3 or 4 disease, involvement of major liver vessels, and low alpha-fetoprotein (AFP) levels 对于PRETEXT 3期或4期、有重要肝血管浸润、甲胎蛋白（AFP）水平较低的患儿，及早由经验丰富的小儿肝外科医师进行治疗非常重要
s334 Sentence The SIOPEL presurgical staging system is used with neoadjuvant chemotherapy followed by definitive surgery, while the COG staging system is based on the findings at time of operation, whenever possible 在术后进行新辅助化疗时多使用SIOPEL术前分期系统，COG分期系统主要基于术中结果
s335 Sentence There are discrepant results on the outcomes for patients with lung metastases at diagnosis who undergo orthotopic liver transplantation following complete resolution of lung disease in response to pretransplant chemotherapy 部分诊断时有肺转移灶的患者在移植前化疗后完全缓解，对于这些患者继续行原位肝移植的预后，不同研究有不同结果
s336 Sentence Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence 儿童与青少年癌症需要多科肿瘤专家医疗团队进行治疗，团队成员需要一定的儿童与青少年肿瘤治疗经验
s337 Sentence Transitional liver cell tumor is a rare neoplasm that is found in older children and adolescents, and has a putative intermediate position between hepatoblasts and more mature hepatocyte-like tumor cells 过渡型肝细胞肿瘤是一种罕见肿瘤，见于年龄较大的儿童和青少年，被认为是肝母细胞与更成熟的类肝细胞肿瘤细胞之间的过渡类型
s338 Sentence Choriocarcinoma of the liver is a very rare tumor that appears to originate in the placenta during gestation and presents with a liver mass in the first few months of life 肝绒毛膜癌是一种极为罕见的肿瘤，为妊娠期胎盘来源，出生后数月出现肝脏肿块
s339 Sentence However, hepatoblastoma has been reported to occur in less than 1% of FAP family members, so ultrasound and AFP screening for hepatoblastoma in members of families with FAP has been controversial 但FAP家族成员仅有不足1%人患有肝母细胞瘤，是否应对其成员进行AFP和超声筛查目前仍有争议
s340 Sentence In a survey of childhood liver tumors treated prior to the consistent use of chemotherapy, only 12 of 33 patients with hepatocellular carcinoma who had complete excision of the tumor survived 一项在持续使用化疗前进行治疗的儿童肝癌患者调查显示，33例肝细胞癌患儿中，只有12例完全切除术后患儿仍然生存
s341 Sentence Hepatoblastoma is also increased in hemihypertrophy, now termed hemihyperplasia, a condition that results in asymmetry between the right and left side of the body when a body part grows faster than normal 肝母细胞瘤风险增高也见于偏侧肥大，现称为偏侧发育过度儿童，即身体的左右部分发育不对称，一部分生长快于正常状态
s342 Sentence The COG is investigating prospective staging of hepatoblastoma patients using the PRETEXT system to determine the timing of surgery and the timing of early notification of liver transplant centers ( COG-AHEP0731 ) COG目前正在研究运用PRETEXT系统对肝母细胞瘤患者进行前瞻性分期对确定手术时间、提前通知肝移植中心时间的意义（COG-AHEP0731）
s343 Sentence Complete surgical resection of the primary tumor continues to be the goal of definitive surgical procedures, but surgical resection is often combined with other treatment modalities (e.g., chemotherapy) to achieve this goal 对原发肿瘤行完全切除一直是外科手术的目标，但目前手术治疗通常还联合其他治疗手段（如：化疗）以期达到这一目标
s344 Sentence For example, in patients with stage I hepatoblastoma at initial diagnosis, aggressive surgical treatment of isolated pulmonary metastases that develop in the course of the disease may make extended disease-free survival possible 比如，对于初诊分期为I期的肝母细胞瘤患者，对孤立性肺转移灶进行积极手术治疗可能延长患者的无病生存期
s345 Sentence Treatment in a clinical trial should be considered if all of the recurrent disease cannot be surgically removed. Phase I and phase II clinical trials may be appropriate and should be considered 对于无法切除的复发灶，应考虑参与临床试验，包括I期或II期临床试验
s346 Sentence This may be due to the additional courses of chemotherapy that are administered before or after resection for patients with stage I and pure fetal histology and after resection for all other patients 对于I期患者、单纯胚胎组织学患者而言，通过切除前、切除后增加化疗疗程，或对于所有其他患者在切除后增加化疗疗程有助于改善预后
s347 Sentence In children and adolescents with primary liver tumors, the surgeon has to be prepared to perform a highly sophisticated liver resection after confirmation of the diagnosis by pathological investigation of intraoperative frozen sections 对于患有原发肝癌的儿童与青少年，小儿外科医师必须有充分心理准备，在病理科医生完成对术中冰冻切片的评估后，小儿外科医师可能需行极其复杂的肝切除术
s348 Sentence In the Children's Oncology Group (COG) study COG-P9645 , 16 patients with stage I pure fetal histology hepatoblastoma with two or fewer mitoses per 10 high power fields were not treated with chemotherapy 儿童肿瘤协作组（COG）的COG-P9645研究募集了16例I期单纯胎儿型肝母细胞瘤患儿，这些患儿组织学检查均为10倍镜下视野中有丝分裂相的细胞小于等于两个，研究中均为给予化疗
s349 Sentence In a European study conducted between 1990 and 1994, 11 patients had tumor found at the surgical margins following hepatic resection and only two patients died, neither of whom had a local recurrence 一项1990至1994年在欧洲进行的研究中，11例肝切除术后的患者切缘阳性，仅2人死亡，这2例患者均无局部复发
s350 Sentence At these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients/families 这些中心提供多种临床试验，囊括了大多数儿童与青少年肿瘤类型，并且对大多数患者和家庭进行公开招募
s351 Sentence Patients whose extrahepatic tumors remain unresectable or who are not transplant candidates should be considered for alternative chemotherapy such as irinotecan, high-dose cisplatin/etoposide, continuous-infusion doxorubicin, radiation therapy, or chemoembolization by hepatic arterial infusion 肝外病变无法切除或未进入肝移植等待列表的患者应当考虑其他治疗，例如伊立替康、大剂量顺铂/阿霉素、连续输注阿霉素、放疗或者经肝动脉化学栓塞术
s352 Sentence A prospective study of 41 patients who were to receive preoperative cisplatin/doxorubicin chemotherapy resulted in some degree of decrease in tumor size with a decrease in AFP level in about 50% of patients 一项对41例患儿的前瞻性研究中，所有患者在术前接受顺铂/阿霉素联合化疗，约有50%的患者肿瘤缩小，伴甲胎蛋白（AFP）水平下降
s353 Sentence Although outcome was nominally higher for children receiving cisplatin/doxorubicin, this difference was not statistically significant, and the combination of cisplatin/vincristine/fluorouracil was significantly less toxic than the doses of cisplatin/doxorubicin, to which it was compared 尽管研究发现使用顺铂/阿霉素联合化疗的患儿预后更好，但其差异不具统计学显著性，且顺铂/长春新碱/氟尿嘧啶联合化疗的毒性显著低于顺铂/阿霉素联合化疗
s354 Sentence The high incidence appears to be related to the incidence of perinatally acquired hepatitis B, which can be prevented in most cases by vaccination and administration of hepatitis B immune globulin to the newborn 这一高发率与围产期感染乙型肝炎病毒相关，多数情况下可以通过对新生儿进行乙肝疫苗接种或乙肝免疫球蛋白注射预防
s355 Sentence On this study, residual metastases are resected at the time of definitive surgery or, in the case of liver transplant, prior to transplantation to render the patient free of extrahepatic disease prior to transplant 该研究中，残留转移灶与主要手术同时进行，或在肝移植术前进行切除残留转移灶以保证患者在移植前无肝外病灶
s356 Sentence A prospective study of 41 patients who were to receive preoperative cisplatin/doxorubicin chemotherapy resulted in some degree of decrease in tumor size with a decrease in alpha-fetoprotein (AFP) levels in about 50% of patients 一项对41例患儿的前瞻性研究中，所有患者在术前接受顺铂/阿霉素联合化疗，约有50%的患者肿瘤缩小，伴甲胎蛋白（AFP）水平下降
s357 Sentence It is important to make the diagnostic distinction between UESL and biliary tract rhabdomyosarcoma because they share some common clinical and pathologic features but treatment differs between the two, as shown in Table 2 UESL和胆道横纹肌肉瘤拥有一些共同的临床与病理特点（如表2所示），但治疗方法各异，因此这两种疾病的鉴别诊断十分重要
s358 Sentence Limited data from a European pilot study suggest that sorafenib may have been beneficial to 12 newly diagnosed patients with advanced hepatocellular carcinoma when given in combination with standard chemotherapy with cisplatin and doxorubicin 欧洲的一项初期研究中，12例新诊断为晚期肝细胞癌的患者同时应用顺铂/阿霉素标准化疗与索拉菲尼，虽然数据有限，但研究认为索拉菲尼使患者获益
s359 Sentence For patients with stage IV disease in which extrahepatic disease is controlled, but the primary tumor remains unresectable following standard chemotherapy, radiation therapy has been used as an interim treatment measure prior to surgical re-exploration 对于分期为IV期、肝外病变得到控制但标准化疗后原发肿瘤无法切除的患者，可在再次尝试外科切除术前使用放射治疗作为过渡
s360 Sentence Thus, it is critically important that a child with probable hepatoblastoma be evaluated by a pediatric surgeon who is experienced in the resection of hepatoblastoma in children and has access to a liver transplant program 因此对于肝母细胞瘤患儿来说，由一位肝母细胞瘤切除术经验丰富的小儿外科医生评估，并有机会加入肝移植项目，对其预后至关重要
s361 Sentence The incidence of hepatocellular carcinoma in the United States is 0.8 in children between the ages of 0 and 14 years and 1.5 in adolescents aged 15 to 19 years per year per 1 million 美国0-14岁儿童中，肝细胞癌的发生率为每百万人0.8例，而15-19岁青少年中则为每百万人1.5例
s362 Sentence Tumor rupture at presentation, resulting in major hemorrhage that can be controlled by transcatheter arterial embolization or partial resection to stabilize the patient, does not preclude a favorable outcome when followed by chemotherapy and definitive surgery 若术中发现肿瘤破裂造成大出血，经导管动脉栓塞止血或部分切除可稳定患者，则在手术和术后化疗的帮助下患者可能仍预后良好
s363 Sentence In the absence of APC germline mutations, childhood hepatoblastomas do not have somatic mutations in the APC gene; however, they frequently have mutations in the beta-catenin gene, the function of which is closely related to APC 当无APC生殖系突变时，儿童肝母细胞瘤也不会有APC基因体突变；但常有β-catenin基因突变，这种基因的功能与APC紧密相关
s364 Sentence Some of these variants do not express INI1 due to INI1 mutation and may be considered rhabdoid tumors of the liver; all small cell undifferentiated hepatoblastomas should be tested for loss of INI1 expression by immunohistochemistry 一些类型的肝母细胞瘤因存在INI1基因突变，使得其不表达INI1，可被认为是肝脏的横纹肌瘤；应当用免疫组化的方法检测所有未分化性小细胞型肝母细胞瘤中INI1的表达
s365 Sentence It should be noted that the Milan criteria for liver transplantation is directed toward adults with cirrhosis and hepatocellular carcinoma. It should not be applied to children and adolescents with hepatocellular carcinoma, especially those without cirrhosis 应当注意，肝移植的米兰标准用于肝硬化和肝细胞癌的成人患者，不应用于肝细胞癌儿童或青少年患者，尤其是无肝硬化的患儿
s366 Sentence Absence of elevated AFP levels at diagnosis occurs in a small percentage of children with hepatoblastoma and appears to be associated with very poor prognosis, as well as with the small cell undifferentiated variant of hepatoblastoma 少数肝母细胞瘤患儿在确诊时AFP水平并不高，这似乎与预后极差相关，也与小细胞未分化型肝母细胞瘤相关
s367 Sentence The 5-year overall survival (OS) in the first international study of hepatoblastoma , in which the study protocol called for treatment of children with preoperative doxorubicin and cisplatin chemotherapy and included children with metastasis, was as follows: 第一项肝母细胞瘤全球研究（包括转移癌患儿）中，儿童术前接受阿霉素和顺铂化疗，研究发现5年生存率如下：
s368 Sentence Some local approaches such as cryosurgery, radiofrequency ablation, and transarterial chemoembolization that suppress hepatocellular carcinoma tumor progression are used as bridging therapy in adults to delay tumor growth while on a waiting list for cadaveric liver transplant 一些局部治疗，如冷冻疗法、射频消融术和经动脉化疗栓塞术均可抑制肝细胞癌的进展，在成人中作为过渡治疗，以帮助延缓肿瘤生长，等待死亡供体肝移植
s369 Sentence Despite these findings, cirrhosis in children, compared with cirrhosis in adults, is much less commonly involved in the development of hepatocellular carcinoma, and is found in only 20% to 35% of livers bearing childhood hepatocellular carcinoma tumors 尽管如此，儿童中的肝硬化则不像成年人一样容易发展为肝细胞癌，只有20%-35%的肝细胞癌患儿有肝硬化
s370 Sentence Because of the poor prognosis in patients with hepatocellular carcinoma, liver transplant should be considered for disorders such as tyrosinemia and familial intrahepatic cholestasis early in the course, prior to the development of liver failure and malignancy 考虑到肝细胞癌的预后较差，应当在酪氨酸血症、家族性肝内胆汁淤积等疾病发现后及早考虑肝移植，防止发展为肝衰竭和肝癌
s371 Sentence The 5-year survival for hepatocellular carcinoma may be dependent on stage; in an Intergroup chemotherapy study conducted in the 1990s, seven of eight stage I patients survived and less than 10% of stage III and IV patients survived 肝细胞癌的5年生存率与肝癌分期相关，20世纪90年代的一项组间化疗对比临床研究中，8名I期患儿中有7名存活，而III期与IV期患儿生存率不足10%
s372 Sentence In the only prospective series from the Italian and German Soft Tissue Sarcoma Cooperative Groups, patients were treated with conservative surgery or biopsy followed by neoadjuvant chemotherapy consisting of varying combinations of vincristine, cyclophosphamide, dactinomycin, doxorubicin, and ifosfamide 唯一一项前瞻性病例分析由意大利与德国软组织肉瘤合作组进行，患者先接受保守性手术或活检术，随后行新辅助化疗，方案包括长春新碱、环磷酸胺、放线菌素、阿霉素和异环磷酰胺的不同组合
s373 Sentence The PRETEXT staging system has a moderate degree of interobserver variability, and the preoperative PRETEXT stage agrees with postoperative pathologic findings only 51% of the time, with overstaging in 37% of patients and understaging in 12% of patients PRETEXT分期系统存在中度的观察者间变异，且仅在51%的情况下，术前与术后的PRETEXT分期相同，有37%的患者分期被高估，而12%的患者分期被低估
s374 Sentence For this reason, completely resected tumors composed of pure fetal histology or of mixed fetal and embryonal cells must have a thorough histologic examination as small foci of undifferentiated small cell histology indicates a need for aggressive chemotherapy 因此，在完全切除单纯胎儿型或混合胎儿型与胚胎组织型肿瘤后，应当对其是否有未分化小细胞灶进行详细的病理学检验，以决定患者是否需要高强度的化疗
s375 Sentence Patients with primary hepatoblastomas that remain unresectable, defined as tumors with less than 1 cm radiographic venous margins, POSTTEXT 3 multifocal or POSTTEXT 4, will be referred to a liver transplant center after the first two cycles of C5VD 对于患有原发性肝母细胞瘤的患者，若放射影像学显示其距静脉边缘不足1cm、POSTTEXT3期多灶性或POSTTEXT4期肿瘤，则无法切除。应考虑行2程C5VD后转入肝移植中心
s376 Sentence Chronic hepatitis B is the leading cause of hepatocellular carcinoma in children in Asian and African countries; however, underlying liver disease can be identified in less than one-third of the children and adolescents with hepatocellular carcinoma in western countries 在亚洲和非洲国家的儿童中，慢性乙肝是导致肝细胞癌发病的首要原因，而在西方国家，患肝细胞癌的儿童或青少年中则只有不到三分之一有慢性乙肝
s377 Sentence In North American clinical trials, the Children's Oncology Group (COG) has recommended that surgery be performed initially if a complete resection can be accomplished (refer to the Postsurgical Staging for Childhood Liver Cancer section of this summary for more information) 在北美临床试验中，儿童肿瘤协作组（COG）建议，在可以行完全手术切除的情况下应当首先考虑行手术切除治疗（更多信息请参见本总结儿童肝癌的术后分期）
s378 Sentence In recent years, virtually all children with hepatoblastoma have been treated with chemotherapy, and in some centers, even children with resectable hepatoblastoma are treated with preoperative chemotherapy, which may reduce the incidence of surgical complications at the time of resection 近年来，几乎所有的肝母细胞瘤患儿均接受化疗。一些治疗中心甚至对可切除肝母细胞瘤患儿行术前化疗，以期减少术中并发症的发生
s379 Sentence Analysis of patients with initially resected hepatoblastoma tumors (prior to receiving chemotherapy) has suggested that those patients with pure fetal histology tumors have a better prognosis than those having an admixture of more primitive and rapidly dividing embryonal components or other undifferentiated tissues 对首先实行肝母细胞瘤切除术（先于化疗）的患儿进行分析，结果显示肿瘤组织学分型为单纯胎儿型的患儿预后较好，优于混合型患儿，后者的肿瘤中含有原始、快速增殖的胚胎成分或其他未分化组织成分
s380 Sentence If the tumor is determined to be unresectable and preoperative chemotherapy is to be administered, it is very important to frequently consult with the surgical team concerning the timing of resection, as prolonged chemotherapy can lead to unnecessary delays and, in rare cases, tumor progression 如果评估认为肿瘤无法完全切除，则需行术前化疗，并且多次与外科医师商议以确定手术时间，因为过长的术前化疗会导致不必要的手术延迟，在一些罕见情况下甚至导致肿瘤的进展
s381 Sentence The improved outcome in older studies may be related to a higher proportion of tumors being less invasive and more resectable in the absence of cirrhosis, because the outcome in recent prospective studies, when compared stage for stage, is not different from other hepatocellular carcinomas 早前的研究报道的良好预后和以下因素相关：多数肿瘤的侵袭性较弱，可切除性更好，无肝硬化。但近期的前瞻性研究显示，相同分期的纤维板层型肝癌与其他肝细胞癌相比，预后并无差异
s382 Sentence This multidisciplinary team approach incorporates the skills of the primary care physician, pediatric surgical subspecialists, radiation therapists, pediatric oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life 这一多科团队需要基础医疗医生、小儿外科专科医生、放疗科医生、小儿肿瘤/血液科医生、康复科医生、小儿护理专家、社工等角色共同构成，以保证儿童能够接受最佳治疗、支持治疗以及康复治疗，尽可能延长生存时间，提高生活质量
s383 Sentence COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer) : Attempted complete surgical resection for all PRETEXT 1 tumors and those PRETEXT 2 tumors with greater than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, or the portal bifurcation COG-AHEP0731（联合化疗治疗新诊断的肝癌患儿）：尝试对所有PRETEXT 1期肿瘤和影像学显示肿瘤边缘距肝中静脉、肝后下腔静脉或门静脉分支超过1cm的PRETEXT 2期肿瘤行完全手术切除
s384 Sentence COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer) : Biopsy only of PRETEXT 2 tumors with less than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, and the portal bifurcation, and of PRETEXT 3 and PRETEXT 4 tumors COG-AHEP0731（联合化疗治疗新诊断的肝母细胞瘤患儿）：对影像学显示肿瘤边缘距肝中静脉、肝后下腔静脉或门静脉分支不足1cm的PRETEXT 2期肿瘤行活检，亦对PRETEXT 3期、PRETEXT4期肿瘤行活检
s385 Sentence Clinical trials in pediatrics are designed to compare potentially better therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment, comparing the results with those previously obtained with standard therapy 儿科的临床试验常将现有的标准治疗与潜在的更好治疗进行对比，而评估这种新治疗的方法包括随机研究设置对照组，或者将新治疗的结果和以往的标准治疗结果相比较
s386 Sentence COG-ADVL1121 (Sorafenib Tosylate in Treating Children With Relapsed or Refractory Rhabdomyosarcoma, Wilms Tumor, Hepatocellular Carcinoma, or Thyroid Cancer) : A phase II study of sorafenib, the Raf kinase and receptor tyrosine kinase inhibitor, in children and young adults with relapsed/refractory rhabdomyosarcoma, Wilms tumor, hepatocellular carcinoma, and papillary thyroid carcinoma COG-ADVL1121（甲苯磺酸索拉菲尼治疗儿童复发性或难治性横纹肌肉瘤、Wilms瘤、肝细胞癌和甲状腺癌）：本研究为一项索拉菲尼II期临床试验。索拉菲尼是一种Raf激酶与酪氨酸激酶受体抑制剂，可用于儿童或青年人的复发性或难治性横纹肌肉瘤、Wilms瘤、肝细胞癌和甲状腺癌
s387 Sentence Gross residual disease/unresected disease non-small cell undifferentiated, AFP level greater than 100 ng/ml (intermediate risk): Two to four cycles of cisplatin/5-fluorouracil/vincristine/doxorubicin (C5VD) followed by resection or orthotopic liver transplant, then an additional two to four cycles of C5VD to total six cycles of chemotherapy, the last two cycles including dexrazoxane 肉眼残留病灶/未切除病灶含小细胞未分化成分，且AFP水平大于100 ng/mL（中等风险）：2-4程顺铂/5-氟尿嘧啶/长春新碱/阿霉素（C5VD），化疗后行切除术或原位肝移植，术后再行2-4程C5VD使总疗程达6程，最后2程加用右雷佐生
s388 Sentence COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer) : Stage IV tumors with any histology and any AFP level or any stage tumor with an AFP level less than 100 ng/ml (high risk): The treatment regimen includes two cycles of vincristine/irinotecan (VI) followed by six cycles of C5VD COG-AHEP0731（联合化疗治疗新诊断的肝母细胞瘤患儿）：任何组织学分型的分期为IV期的肿瘤，不论AFP水平，或是任意分期且AFP水平小于100 ng/mL的肿瘤（高危）：治疗方案为2程长春新碱/伊立替康（VI）联合化疗，加6程C5VD
s389 Sentence The United Network for Organ Sharing (UNOS) Standard Transplant and Research Files registry reported all children younger than 18 years listed for a liver transplant in the United States from October 1987 through July 2004. Of these children, 135 had hepatoblastoma and 41 had hepatocellular carcinoma and both groups received liver transplant with 5-year survival rates of 69% for hepatoblastoma and 63% for hepatocellular carcinoma 美国器官资源共享中心（UNOS）标准移植与研究文件记录了全美从1987年10月到2004年7月所有18岁以下等待肝移植的儿童，其中有135名儿童患有肝母细胞瘤，41名儿童患有肝细胞癌，肝母细胞瘤组和肝细胞癌组患儿接受肝移植后的5年生存率分别是69%和63%