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

• Published in: Annals of surgery Vol. 231; no. 1; pp. 74 - 81
• Main Authors: CHICHE, L, THONG DAO, SALAME, E, GALAIS, M. P, BOUVARD, N, SCHMUTZ, G, ROUSSELOT, P, BIOULAC-SAGE, P, SEGOL, P, GIGNOUX, M
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott 2000
• Subjects: Adenoma, Liver Cell - genetics; Adenoma, Liver Cell - pathology; Adenoma, Liver Cell - surgery; Adolescent; Adult; Biological and medical sciences; Cell Transformation, Neoplastic - pathology; Female; Gastroenterology. Liver. Pancreas. Abdomen; Hepatectomy; Humans; Liver - pathology; Liver Neoplasms - genetics; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Liver Transplantation; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Middle Aged; Original; Pedigree; Tomography, X-Ray Computed; Treatment Outcome; Tumors; Human; Liver; Hepatic disease; Case study; Pathology; Symptomatology; Adenomatosis; Treatment; Surgery; Digestive diseases; Evolution; Medical imagery; Benign neoplasm; Diagnosis; Bibliographic review
• ISSN: 0003-4932; 1528-1140
• DOI: 10.1097/00000658-200001000-00011

Liver adenomatosis (LA) is a rare disease originally defined by Flejou et al in 1985 from a series of 13 cases. In 1998, 38 cases were available for analysis, including eight personal cases. The aim of this study was to review and reappraise the characteristics of this rare liver disease and to discuss diagnosis and therapeutic options. LA was defined as the presence of >10 adenomas in an otherwise normal parenchyma. Neither female predominance nor a relation with estrogen/progesterone intake has been noted. Natural progression is poorly known. The clinical presentation, evolution, histologic characteristics, and therapeutic options and results were analyzed based on a personal series of eight new cases and an updated review of the literature. From a diagnostic standpoint, two forms of liver adenomatosis with different presentations and evolution can be defined: a massive form and a multifocal form. The role of estrogen and progesterone is reevaluated. The risks of hemorrhage and malignant transformation are of major concern. In the authors' series, liver transplantation was indicated in two young women with the massive, aggressive form, and good results were obtained. Liver adenomatosis is a rare disease, more common in women, where outcome and evolution vary and are exacerbated by estrogen intake. Most often, conservative surgery is indicated. Liver transplantation is indicated only in highly symptomatic and aggressive forms of the disease.