Hepatic Adenomatosis in a Young Asymptomatic but High-risk Patient: An Incidental Diagnosis with Potentially Severe Clinical Implications

Hepatic adenomatosis (HA) is a very rare condition and defined as the presence of 10 or more adenomas in an otherwise normal liver. HA has an incidence of 10–24% in patient with hepatic adenoma and it is more common in women. Most patients with HA are asymptomatic with a normal liver function test and half of cases are detected incidentally on imaging. Although HA is considered a benign disease, some patients may develop potentially fatal complications, such as hypovolaemic shock due to rupture of the liver lesion or malignant transformation to hepatocellular carcinoma. We report the case of a 29-year-old woman who presented to the emergency room after a car accident. Whole-body computed tomography revealed multiple focal hepatic hypervascular lesions in the right lobe of the liver together with a fatty liver. Subsequent hepatic magnetic resonance imaging suggested the diagnosis of HA with a suspicion of focal nodular hyperplasia (FNH). The patient refused to undergo liver biopsy, so we instituted a 3-month surveillance program, which included clinical assessment, liver function tests, tumour marker assessment and blood tests as well as sonographic evaluation for follow-up of the liver lesions.

Coordinated Cross-Talk Between the Myc and Mlx Networks in Liver Regeneration and Neoplasia

The Myc bHLH-ZIP transcription factor is deregulated by most cancers. As a heterodimer with the bHLH-ZIP protein Max, Myc regulates target genes that contribute to metabolism and proliferation. This "Myc Network" cross-talks with the "Mlx Network" comprised of the Myc-like bHLH-ZIP proteins MondoA and ChREBP and the Max-like bHLH-ZIP protein Mlx. This "Extended Myc Network" regulates genes with both common and distinct functions. We have generated hepatocytes lacking Mlx (mlxKO) or Mlx+Myc (double KO or DKO) and quantified their abilities to replace dying hepatocytes in a murine model of Type I tyosinemia. We find that this function deteriorates as the Extended Myc Network is progressively dismantled. Genes dysregulated in mlxKO and DKO hepatocytes include those involved in translation and mitochondrial function. The Myc and Mlx Networks thus cross-talk with the latter playing a disproportionate role. mycKO and mlxKO mice also develop non-alcoholic fatty liver disease and mlxKO and DKO mice develop extensive hepatic adenomatosis not observed in wild-type, mycKO, chrebpKO or mycKOxchrebpKO mice. In addition to demonstrating cooperation between the Myc and Mlx Networks, this study reveals the latter to be more important in maintaining metabolic and translational homeostasis, while concurrently serving as a suppressor of benign tumorigenesis.

From an Incidental Finding to an Emergent Treatment: A Case Report of a Hepatic Adenomatosis and Large Ruptured Hepatic Adenoma

Introduction. Hepatic adenoma is an uncommon benign liver tumor presenting as solitary lesions or even rarely as hepatic adenomatosis. Large lesions carry a risk of rupture, hemorrhage, and malignant transformation. This case report aims to increase awareness about risk factors for hepatic adenomas, considering the increasing prevalence of obesity and the widespread use of oral contraceptive pills. Case Presentation. A 20-year-old obese female who was taking oral contraceptive pills for seven years presented to the emergency department with vomiting and abdominal pain caused by gastroenteritis. On imaging, multiple hepatic adenomas, including two lesions 6 and 9 cm in diameter, were incidentally found. During the hospitalization, the patient suddenly developed acute anemia and rupture of the largest lesion, which was promptly treated with arterial embolization. Discussion. Obesity and exposure to hormones are well-known risk factors for hepatic adenomas. The incidence of hepatic adenomas is steadily increasing because of the prevalence of obesity, especially among females. Lifestyle interventions for weight loss and discontinuation of oral contraceptive pills are considered a conservative treatment of hepatic adenomas. Large lesions possess the risk of malignant transformation and rupture and require surgical excision.

Liver Transplantation for Hepatic Adenomatosis: The First Case Report in Thailand and Literature Review

Hepatic adenomas are benign solid liver tumors commonly found in young females and usually asymptomatic. Hepatic adenomatosis is characterized by more than 10 adenomas in an otherwise normal liver. Bleeding and malignant transformation uncommonly occur especially in tumor larger than 5 cm and in male patients. The authors reported a case of a young female with large multiple hepatic adenomas from hepatic adenomatosis that presented with abdominal pain, large abdominal mass, weight lost, and abnormal liver function tests. She failed transarterial embolization. Surgical resection was not offered due to extent of the hepatic adenomas. She received orthotopic liver transplantation as rescue therapy. Her explanted liver showed multiple foci of hepatocellular carcinoma (HCC). Her post-operative was uneventful. The authors reported a case of liver transplantation for the treatment of unresectable hepatic adenomatosis. Keywords: Hepatic adenomatosis, Malignant transformation, Liver transplantation

Hepatic Adenomatosis in Aicardi Syndrome: A Clinical Report and Review of The Literature

Aicardi Syndrome is a rare X-linked dominant genetic disorder characterized by callosal agenesis, generalized seizures, chorioretinal lacunae and vertebral anomalies. Uncommon neoplasms have been previously observed in affected patients. We describe the case of a 19-year-old woman with Aicardi Syndrome developing multiple giant mass lesions in the liver. Histopathology revealed hepatic adenomas.

Hepatic Adenomatosis: Diagnosis and Management Dilemma

Hepatic adenomatosis is a rare, benign tumour of the liver. It was first described by Flejou et al as multiple adenomas in an otherwise normal liver parenchyma. Although benign, it can present as a diagnostic challenge because the lesions can be difficult to distinguish from other hepatic tumours. Patients can be asymptomatic and the diagnosis may only be made incidentally. We describe the case of 40-year-old Malay lady who was incidentally found to have hepatomegaly. Radiological examinations revealed a complex left ovarian cyst with multiple liver lesions. Biopsy of the liver lesion showed features of hepatic adenomatosis. Literature review was done and the dilemma in managing her was discussed.