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肝母细胞瘤的最新进展:病理学、遗传学、风险分层及化疗

Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy.

作者信息

Czauderna Piotr, Lopez-Terrada Dolores, Hiyama Eiso, Häberle Beate, Malogolowkin Marcio H, Meyers Rebecka L

机构信息

aDepartment of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland bDepartment of Pathology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas, USA cDepartment of Surgery, Natural Science Center for Basic Research and Development, Hiroshima University Hospital, Hiroshima, Japan dChildren's Hospital, Ludwig-Maximilians-University, Munich, Germany eDepartment of Pediatric Hematology and Oncology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin fDepartment of Pediatric Surgery, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah, USA.

出版信息

Curr Opin Pediatr. 2014 Feb;26(1):19-28. doi: 10.1097/MOP.0000000000000046.

Abstract

PURPOSE OF REVIEW

As a rare pediatric tumor, hepatoblastoma presents challenges to the individual practitioner as no center will see more than a handful of cases each year.

RECENT FINDINGS

The Children's Hepatic tumor International Collaborative (CHIC) effort has fostered international cooperation in this rare children's tumor, leading to the establishment of a large international collaborative dataset, the CHIC database, which has been interrogated to refine risk stratification and inform treatment options. Apace with this effort has been the international collaboration of pediatric pathologists working together to establish a new international histopathologic consensus classification for pediatric liver tumors as a whole, with particular focus on the histological subtypes of hepatoblastoma.

SUMMARY

International collaborative efforts in hepatoblastoma have led to a new international histopathologic consensus classification, refinements in risk stratification, advances in chemotherapy, and a better understanding of surgical resection options forming the foundation for the development of an upcoming international therapeutic trial.

摘要

综述目的

肝母细胞瘤作为一种罕见的儿科肿瘤,给个体从业者带来了挑战,因为每年没有哪个中心会见到超过少数几例病例。

最新发现

儿童肝脏肿瘤国际协作组(CHIC)的工作促进了在这种罕见儿童肿瘤方面的国际合作,促成了一个大型国际协作数据集即CHIC数据库的建立,该数据库已被用于完善风险分层并为治疗方案提供依据。与此同时,儿科病理学家开展国际合作,共同为整个儿童肝脏肿瘤建立一种新的国际组织病理学共识分类,尤其关注肝母细胞瘤的组织学亚型。

总结

在肝母细胞瘤方面的国际协作努力带来了一种新的国际组织病理学共识分类、风险分层的细化、化疗的进展以及对手术切除方案的更好理解,为即将开展的国际治疗试验奠定了基础。

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