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 Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate.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. 1. Introduction Liver tumors are very rare in children, accounting for 1 to 4% of all pediatric tumors. Benign tumors account for 30 to 40% of these, with a majority of hemangiomas occurring during infancy. Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCAs) are extremely rare during childhood, and there are few published reported cases and series. Presentation, physiopathology, and management differ from adults. We will successively review the main characteristics of FNH and HCA in children and discuss physiopathology, followup, and therapeutic modalities based on a systematic review of the literature and our experience. 2. Focal Nodular Hyperplasia 2.1. Histological Definition and Physiopathology Focal nodular hyperplasia (FNH) is not a neoplasm but a nonspecific hyperplasic reaction to vascular abnormalities. It is a well-delimitated lesion without capsules and characterized by hepatocytic nodules separated by fibrous bands. The mass has a central stellate fibrous region containing malformed vascular structures that include large arteries, without portal veins. Bile ductular reaction is usually present at the interface between hepatocytes and fibrous bands and is highly suggestive of the diagnosis of FNH. According to some authors, in FNH, arterial blood flows from the anomalous arteries via capillaries into sinusoids adjacent to the
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