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International Journal of Hepatology
Volume 2013, Article ID 215064, 11 pages
http://dx.doi.org/10.1155/2013/215064
Review Article

Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma

1Department of Pediatric Radiology, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 Rue du General Leclerc, 94278 Le Kremlin-Bicêtre, France
2Department of Pediatric Surgery, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 Rue du General Leclerc, 94278 Le Kremlin-Bicêtre, France

Received 9 November 2012; Accepted 5 February 2013

Academic Editor: Paulette Bioulac-Sage

Copyright © 2013 Stéphanie Franchi-Abella and Sophie Branchereau. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Benign liver tumors are very rare in children. Most focal nodular hyperplasia (FNH) remain sporadic, but predisposing factors exist, as follows: long-term cancer survivor (with an increasing incidence), portal deprivation in congenital or surgical portosystemic shunt. The aspect is atypical on imaging in two-thirds of cases. Biopsy of the tumor and the nontumoral liver is then required. Surgical resection will be discussed in the case of large tumors with or without symptoms. In the case of associated vascular disorder with portal deprivation, restoration of the portal flow will be discussed in the hope of seeing the involution of FNH. HepatoCellular Adenoma (HCA) is frequently associated with predisposing factors such as GSD type I and III, Fanconi anemia especially if androgen therapy is administered, CPSS, and SPSS. Adenomatosis has been reported in germline mutation of HNF1-α. Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate. Surgery is usually performed on large lesions. In the case of adenomatosis or multiple lesions, surgery will be adapted. Close followup is required in all cases.