Polymorphic multiple hepatocellular adenoma including a non-steatotic HNF1α-inactivated variant
Kayvan Mohkam, Benjamin Darnis, Jean-Baptiste Cazauran, Agnès Rode, Anne-Frédérique Manichon, Christian Ducerf, Brigitte Bancel and Jean-Yves Mabrut
Department of General Surgery and Liver Transplantation (Mohkam K, Darnis B, Cazauran JB, Ducerf C and Mabrut JY), Department of Radiology (Rode A and Manichon AF), Department of Surgical Pathology (Bancel B), Hospices Civils de Lyon, Croix-Rousse University Hospital, Lyon, France; Equipe Mixte de Recherche 3738, Ecole Doctorale EDISS 205, Université Lyon 1, France (Mohkam K, Darnis B and Mabrut JY)
Corresponding Author: Kayvan Mohkam
(Email: kayvan.mohkam@chu-lyon.fr)
Abstract：To the Editor:
Hepatocellular adenomas (HCAs) consist of benign liver tumors favored by the use of oral contraceptives, which preferentially occur in women.[1, 2] They expose to the risk of hemorrhage (20% of cases) and more rarely, to the risk of malignant transformation (4%-10% of cases).[3, 4] Multiple HCAs, which are defined by the presence of 10 or more HCAs, were first described in 1985 and were initially considered having a worse prognosis than single HCA.[5] However, recent reports have suggested that the risk of complications in patients with multiple HCAs was not related to the number of lesions, but mainly to three other factors: i) tumor size >5 cm for the risk of both hemorrhage and malignant transformation;[3] ii) male gender and iii) β-catenin-mutation for the risk of malignant transformation.[6] Progresses made in terms of genotypic knowledge of HCA have allowed to identify different subtypes of HCA associated with more specific risks of complication.[7] A recent report has suggested that some patients could present with mixed subtypes of HCA.[8] Herein, we report the case of a female patient presenting with polymorphic multiple HCA of three different subtypes, including a very unusual non-steatotic variant of hepatocyte nuclear factor 1 homeobox α (HNF1α)-inactivated HCA mimicking malignant transformation, which was also complicated with repeated episodes of hemorrhage and was finally managed with liver transplantation.
2017, Vol. 16 
