Liver adenomatosis: reappraisal, diagnosis, and surgical management: eight new cases 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. 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.

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Figure 1. Necropsis: liver specimen weighing 4,200 g. The liver is deformed by huge nodules. On the right, rupture of one adenoma was responsible for intraperitoneal hemorrhage and death.

Figure 2. Macroscopic view of liver adenomatosis. Note the nodules of different size with hemorrhagic areas.

Figure 3. Computed tomography scan showing the central adenoma with intratumoral hemorrhage and another large subcapsular nodule in the left lobe.

Figure 4. Pseudoacinar arrangement of hepatocytes made us suspect degeneration.

Figure 5. Genealogic tree of patients 1 to 4. Circles or boxes with a slash indicate that that person is dead; #, hepatic hemorrhage after minimal trauma (further data not available); US*, one hyperechoic nodule detected on ultrasound; USN, ultrasound considered normal; IDD, insulin-dependent diabetes; NIDD, noninsulin-dependent diabetes.

Figure 6. Adenomatous hyperplasia. The cells can be distinguished by lighter cytoplasm. Hematoxylin and eosin ×40.