Hepatic adenomatosis in an Hispanic patient. A case report and review of the literature

Abstract

Objective: Liver adenomatosis (LA) is a rare disease originally defined by Flejou et al. in 1985 from a series of 13 cases. Only 57 cases have been reported in the literature, and all have been documented among Caucasian population. The aim of this study is to review and reappraise the characteristics of this rare liver disease, and to discuss the diagnosis and therapeutic options. Background: LA is defined as the presence of >10 adenomas in an otherwise normal liver parenchyma. Neither female predominance nor a relation with estrogen/progesterone intake has been noted. Natural progression is poorly understood. Methods: We describe the clinical presentation, evolution, radiologic studies, histologic characteristics and therapeutic options in a 3rd generation Mexican woman with LA. We also include an updated review of the literature. Results: The natural history and pathogenesis of LA are unclear. The risk of spontaneous hemorrhage or malignant transformation are a major concern. There is controversy regarding the optimal treatment for this disease; treatment options range from conservative medical therapy to surgical resection and even liver transplantation. Conclusion: LA is a rare disease, more common in women, and its outcome and evolution vary. Most often, conservative surgery is indicated. Liver transplantation is indicated only in highly symptomatic and aggressive forms of the disease.