Liver adenomatosis: Reappraisal, diagnosis, and surgical management - Eight new cases and review of the literature

Objective Liver adenomatosis (LA) is a rare disease originally defined by Flejou et a l in 1985 from a series of 13 cases. In 1998, 38 cases were available for a nalysis, including eight personal cases. The aim of this study was to revie w and reappraise the characteristics of this rare liver disease and to disc uss diagnosis and therapeutic options. Background LA was defined as the presence of >10 adenomas in an otherwise normal paren chyma. Neither female predominance nor a relation with estrogen/progesteron e intake has been noted. Natural progression is poorly known. Methods The clinical presentation, evolution, histologic characteristics, and thera peutic options and results were analyzed based on a personal series of eigh t new cases and an updated review of the literature. Results From a diagnostic standpoint, two forms of liver adenomatosis with differen t presentations and evolution can be defined: a massive form and a multifoc al form. The role of estrogen and progesterone is reevaluated. The risks of hemorrhage and malignant transformation are of major concern. In the autho rs' series, liver transplantation was indicated in two young women with the massive, aggressive form, and good results were obtained. Conclusion Liver adenomatosis is a rare disease, more common in women, where outcome a nd evolution vary and are exacerbated by estrogen intake. Most often, conse rvative surgery is indicated. Liver transplantation is indicated only in hi ghly symptomatic and aggressive forms of the disease.