小儿亲体部分肝移植治疗肝母细胞瘤(附1例报告)

目的探讨小儿部分亲体肝移植治疗肝母细胞瘤的临床经验。方法对1例患肝母细胞瘤的4岁男童实施母亲供肝,左外肝叶亲体部分肝移植。结果供体于术后7d痊愈出院,受体于术后22d出院,无并发症。随访24个月,小儿生长发育良好,正常上学,无特殊不适。结论实施小儿亲体部分肝移植扩大了供肝来源,临床疗效好,可作为治疗肝母细胞瘤等小儿终末期肝病的有效治疗方法。     

幼儿肝母细胞瘤的多学科综合治疗体会

[摘 要] 目的 探讨多学科协助模式在幼儿肝母细胞瘤诊治中的应用价值。方法 回顾性分析2014年4月至2017年5月湖南省人民医院收治的8例经多学科综合诊治的肝母细胞瘤患儿的临床资料。观察指标：（1）治疗前病变范围（PRETEXT）分期、手术情况;（2）病理组织学特点,术后化疗方案;（3）随访患儿中位生存时间、无瘤生存率、总体生存时间。采用门诊和电话方式进行随访。结果 8例患儿均经过多学科讨论,术前PRETEXT分期,I期1例,II期3例,III期3例,IV期1例;术后病理结果,3例为单纯胎儿型肝母细胞瘤,5例为混合型肝母细胞瘤。术后1个月开始接受顺铂联合多柔比星化疗,共4个周期。随访时间4～40个月,中位随访时间为21.5个月,所有患儿均为无瘤生存。结论 根治性手术切除是治疗儿童肝母细胞瘤的基础,多学科综合诊治可为肝母细胞瘤患儿制定个体化治疗方案。     

Congenital Hepatoblastoma and Beckwith-Wiedemann Syndrome: A Case Study Including DNA Ploidy Profiles of Tumor and Adrenal Cytomegaly

A case of fatal congenital hepatoblastoma is described in which the autopsy provided the first evidence of Beckwith-Wiedemann syndrome. Aneuploid quantitative DNA patterns were found by image analysis of the tumor and the cytomegalic adrenal gland.     

Chromosome Rearrangement with No Apparent Gene Mutation in Familial Adenomatous Polyposis and Hepatocellular Neoplasia

We have identified a constitutional inversion in chromosome 5 associated with familial adenomatous polyposis in three generations of a Mexican family. Two of three siblings developed hepatic neoplasia in infancy. The gene truncation assay failed to demonstrate a truncated protein in the segment harboring the adenomatous polyposis coli (APC) genes. Polymerase chain reaction (PCR) amplification of APC gene coding exons and sequencing of PCR products did not reveal any significant mutation. The data suggest that in this family, the phenotype may be the result of a “position effect.” Received May 21, 2001; accepted July 23, 2001.     

Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked overgrowth syndrome associated with multiple congenital anomalies caused by a mutant X-linked recessive trait. The authors report on a 14-month-old male patient affected by hepatoblastoma. As far as is known, this is the second patient reported with SGBS and hepatoblastoma. The observations emphasize that an increased risk of neoplasia in SGBS must be kept in mind, especially in young patients.     

Parental cigarette smoking and childhood risks of hepatoblastoma: OSCC data

Reported cigarette smoking habits for the parents of 43 UK children who died with hepatoblastoma (1953-55 deaths, 1971-81 deaths) have been compared with corresponding information for the parents of 5777 healthy control children by means of unconditional logistic regression. Hepatoblastoma risks were doubled if both parents smoked relative to neither parent smoking (RR 2.28, 95% CI 1.02-5.09).