Hepatic Adenomatosis: A Rare Clinical Entity with Unique Characteristics

Abstract

Introduction: Hepatic adenomatosis (HA) is a rare entity. Complications can ensue and may be related to hemorrhage, excessive growth with pain and malignancy. We present a case in a 33-year old female suffering from persistent growth of HA despite past surgical intervention. Case: Caucasian female presents with right upper quadrant tenderness (RUQ), nausea, and vomiting worsening over three days. Past history significant for a right lobectomy and left lateral segmentectomy of the liver after a fall 20 years earlier. The patient used oral contraceptives for less than a year as a teenager. On exam, she was tachycardic and hypotensive. Abdomen was distended, firm, RUQ tenderness, and hepatomegaly. Bowel sounds were present. Laboratory data revealed H&H 12/36, AST 216 U/L, ALT 216 U/L, albumin 3.8 g/dl, alkaline phosphatase 138 U/L, INR 1.1, AFP 1.5 ng/mL, and lactic acid 2.9 mmol/L. Initial CT scan demonstrated extravasation of contrast in the caudate lobe suggestive of a bleed. Angiogram showed an irregular branch of the hepatic artery to the caudate lobe which was embolized. Repeat CAT scan revealed multiple hepatic lesions and hemorrhage (subcapsular, retroperitoneal, and intraperitoneal). Her condition worsened thus she was listed and received a cadaveric liver transplant. The explanted liver showed presence of a 17×10×5 cm hematoma in the liver. It also contained 50–100 adenomas ranging in size from 0.5 cm–4.5 cm. Pathology revealed hepatic adenomatosis. Discussion: Hepatic adenomatosis is a rare entity characterized by greater then 10 adenomas. Unlike solitary hepatic adenoma, there is lack of female predominance or association with glycogen storage diseases. Lesions do not have a tendency towards influence by steroid or estrogen use. The etiology is poorly understood and is associated with congenital or acquired abnormalities of hepatic vasculature. Clinically, these lesions are more painful and result in significant hepatomegaly. Bleeding and malignant transformation occurs in 62% and 13% of cases respectively. Due to the risk of hemorrhage and malignancy, resection of the largest and most vulnerable lesion is often required. Surgical resection has been shown to decrease abdominal pain and lead to longer hemorrhage free intervals and need close surveillance. Patients who progress despite radiological and surgical interventions require liver transplantation.