What we have to know about paediatrics hepatoblastoma

被引:0
|
作者
Taque, S. [1 ]
Buendia, M. A. [2 ]
Fabre, M. [3 ]
Branchereau, S. [4 ]
Pariente, D. [5 ]
Bruneau, B. [6 ]
Brugieres, L. [7 ]
机构
[1] CHU Hop Sud, Serv Hemato Oncol Pediatr, 16,Blvd Bulgarie, F-35203 Rennes, France
[2] Hop Paul Brousse, Ctr Hepatobiliaire, Inserm U785, F-94800 Villejuif, France
[3] Inst Gustave Roussy, Serv Pathol Morpholog, F-94805 Villejuif, France
[4] CHU Bicetre, Dept Chirurg, F-94270 Le Kremlin Bicetre, France
[5] CHU Bicetre, Serv Radiol Pediatr, F-94270 Le Kremlin Bicetre, France
[6] CHU Hop Sud, Serv Radiol & Imagerie Med, F-35203 Rennes, France
[7] Inst Gustave Roussy, Dept Pediat, F-94805 Villejuif, France
来源
关键词
Liver tumor; Hepatoblastoma; Alfa-foetoprotein; SIOPEL;
D O I
10.1016/J.oncohp.2013.10.003
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Hepatoblastoma is the most common hepatic tumor in paediatric population with an increasing incidence. It occurs almost in patients younger than 3 years without history of liver disease. Even if the aetiology is unknown, it can be associated with a congenital abnormality or an APC mutation. Hepatoblastoma appears as a well-circumscribed liver mass with, in most cases, an abnormal elevation of alfa-foetoprotein level (AFP). The imaging characteristics of the tumor reflect its gross pathologic appearance and histological composition. A biopsy is mandatory to confirm the diagnosis and study biological markers of the tissue. The radiological characteristics and the histology have permitted to build a new classification and a stratification of the treatment. Cisplatinum associated with surgical resection is the mainstay of treatment. Tumors considered unresectable may be treated with liver donor transplantation. The intensification of the chemotherapy and the radical surgery have increased the complete resection rate and the overall survival (80%) even in the high risk stage. (C) 2013 Elsevier Masson SAS. All rights reserved.
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页码:111 / 119
页数:9
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