Franchi-Abella Stéphanie, Branchereau Sophie
Department of Pediatric Radiology, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 Rue du General Leclerc, 94278 Le Kremlin-Bicêtre, France.
Int J Hepatol. 2013;2013:215064. doi: 10.1155/2013/215064. Epub 2013 Mar 11.
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 Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate.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.
良性肝肿瘤在儿童中非常罕见。大多数局灶性结节性增生（FNH）为散发性，但存在易感因素，如下：长期癌症幸存者（发病率呈上升趋势）、先天性或手术性门体分流导致的门静脉缺失。三分之二病例的影像学表现不典型。此时需要对肿瘤及非肿瘤性肝脏进行活检。对于有症状或无症状的大肿瘤，将讨论手术切除。对于伴有门静脉缺失的相关血管疾病，将讨论恢复门静脉血流以期观察FNH的消退情况。肝细胞腺瘤（HCA）常与易感因素相关，如I型和III型糖原贮积病、范可尼贫血（尤其是接受雄激素治疗时）、先天性肝门脉分流（CPSS）和肝内门体分流（SPSS）。已报道在肝细胞核因子1α（HNF1-α）的种系突变中出现腺瘤病。治疗将取决于易感因素的存在情况，可能包括代谢控制、停用雄激素治疗或在适当的时候关闭分流。通常对大的病变进行手术。对于腺瘤病或多发病变，手术方式将进行调整。所有病例均需密切随访。
