Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma
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 imagi...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | International Journal of Hepatology |
| Online Access: | http://dx.doi.org/10.1155/2013/215064 |
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| author | Stéphanie Franchi-Abella Sophie Branchereau |
| author_facet | Stéphanie Franchi-Abella Sophie Branchereau |
| author_sort | Stéphanie Franchi-Abella |
| collection | DOAJ |
| description | 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 Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate.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. |
| format | Article |
| id | doaj-art-c8e1369d5d114ee5a9555e4471cd2798 |
| institution | Kabale University |
| issn | 2090-3448 2090-3456 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Hepatology |
| spelling | doaj-art-c8e1369d5d114ee5a9555e4471cd27982025-08-20T03:24:11ZengWileyInternational Journal of Hepatology2090-34482090-34562013-01-01201310.1155/2013/215064215064Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular AdenomaStéphanie Franchi-Abella0Sophie Branchereau1Department of Pediatric Radiology, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 Rue du General Leclerc, 94278 Le Kremlin-Bicêtre, FranceDepartment of Pediatric Surgery, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 Rue du General Leclerc, 94278 Le Kremlin-Bicêtre, FranceBenign 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.http://dx.doi.org/10.1155/2013/215064 |
| spellingShingle | Stéphanie Franchi-Abella Sophie Branchereau Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma International Journal of Hepatology |
| title | Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma |
| title_full | Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma |
| title_fullStr | Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma |
| title_full_unstemmed | Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma |
| title_short | Benign Hepatocellular Tumors in Children: Focal Nodular Hyperplasia and Hepatocellular Adenoma |
| title_sort | benign hepatocellular tumors in children focal nodular hyperplasia and hepatocellular adenoma |
| url | http://dx.doi.org/10.1155/2013/215064 |
| work_keys_str_mv | AT stephaniefranchiabella benignhepatocellulartumorsinchildrenfocalnodularhyperplasiaandhepatocellularadenoma AT sophiebranchereau benignhepatocellulartumorsinchildrenfocalnodularhyperplasiaandhepatocellularadenoma |