Ruptured hepatic adenoma in liver adenomatosis: a case report of emergency surgical management.

In hepatic adenomatosis, multiple liver cell adenomas (usually > or = 10) generally affects patients with no prior history of oral contraceptive use, androgenic steroid use, or glycogen storage disease. We report a rare case of a 44 year-old female who underwent emergency surgery for hemoperitoneum due to spontaneous rupture of a liver cell adenoma in hepatic adenomatosis, after prolonged use of two different contraceptives (Gestodene and Ethinylestradiol).     

Two cases of hepatocellular adenomatosis treated with transcatheter arterial embolization

Hepatocellular adenoma is a rare benign tumor of the liver. However, some complications, such as hemorrhage, rupture, and malignant transformation, have been reported previously. Surgical resection is considered to be the best choice of treatment, when adenomas are increasing in size, while resection is difficult to perform when multiple adenomas develop throughout the liver. Here, we report two cases of multiple hepatocellular adenomatosis. One patient had a history of aplastic anemia and the other had glycogen storage disease. We treated them with transcatheter arterial embolization (TAE) to prevent hemorrhage and rupture. After TAE, most parts of the adenomas showed necrotic change. These cases suggest that TAE is an effective treatment of hepatocellular adenomatosis.     

A CASE OF HEPATOCELLULAR ADKNOMATOSIS WITH A FOLLOW-UP OF 11 YEARS

Hepatoccllular adenomatosis is characterized by the presence of numerous (arbitrarily > 10) adenomas within an otherwise normal liver without a history of glycogen storage disease or steroid hormone therapy. Although the disease is rare, its importance lies in its tendency to produce symptoms such as abdominal pain and its potential for abdominal hemorrhages. However, the prognosis of hepatocellular adenomatosis remains uncertain. Here we describe the ease of a -40-yr-old female with hepatoccllular adenomatosis without evidence of serious complications, who was observed over a period of 11 yr.     

Liver cell adenoma and liver cell adenomatosis

During the last three decades liver cell adenoma and liver cell adenomatosis have emerged as new clinical entities in hepato-logical practice due to the widespread use of oral contraceptives and increased imaging of the liver. On review of published series there is evidence that 10% of liver cell adenomas progress to hepatocellular carcinoma, diagnosis is best made by open or laparoscopic excision biopsy, and the preferred treatment modality is resection of the liver cell adenoma to prevent bleeding and malignant transformation. In liver cell adenomatosis, the association with oral contraceptive use is not as high as in solitary liver cell adenomas. The risk of malignant transformation is not increased compared with solitary liver cell adenomas. Treatment consists of close monitoring and imaging, resection of superficially located, large (>4 cm) or growing liver cell adenomas. Liver transplantation is the last resort in case of substantive concern about malignant transformation or for large, painful adenomas in liver cell adenomatosis after treatment attempts by liver resection.     

Synchronous bleeding of liver adenomatosis and possible relation to acoustic trauma

BackgroundSolitary hepatic adenoma is a rare tumour but adenomatosis of the liver is even less common; it has been defined arbitrarily as more than four adenomas within otherwise normal hepatic parenchyma. Usually asymptomatic, the main clinical presentation is abdominal pain secondary to bleeding from the tumour.Case outlineA 40-year-old woman admitted for abdominal pain and operated within a few hours due to circulatory instability was found to have synchronous bleeding from three out of four liver cell adenomas. The onset of pain followed shortly after exposure to particularly loud music. The three bleeding tumours were enucleated. The fourth lesion was left in situ; during outpatient follow-up it decreased in size and eventually disappeared.DiscussionAlthough rupture with bleeding is a well-known presentation of liver cell adenomas, synchronous bleeding from more than one tumour is exceedingly uncommon and this may be the first reported case. Enucleation of the tumour is easy to perform even during active bleeding. Small asymptomatic tumours may be followed without surgical intervention.     

Hepatic adenomatosis--a rare double complication of multiple adenoma rupture and malignant transformation.

Hepatic adenomatosis (HA) is a rare disorder that is susceptible to hemorrhagic complications and, rarely, to malignant transformation. We report a 24-year-old woman who was found to have HA with > 10 tumors; she presented with simultaneous rupture of two of the adenomas, along with malignant change in one. Hematoma evacuation and caudate lobe resection were done and she has been doing well 6 months later.     

MR findings in liver adenomatosis

Liver adenomatosis is a rare entity in which multiple hepatic adenomas (more than 10) occur in patients with no prior history of oral contraceptives or androgenic steroid use. This report describes findings of this entity on magnetic resonance imaging (MRI) and discusses the differential diagnosis of this disease.     

Liver Cell Adenoma: Diagnosis and Treatment of a Rare Hepatic Neoplastic Process

Liver adenomatosis is a rare entity in which multiple liver cell adenomas (more than 10) occur in patients with no prior history of steroid use or glycogen storage disease. This report describes a case of liver adenomatosis, distinguishes liver adenomatosis from other benign liver lesions, and discusses the diagnosis and treatment of this disorder.     

Duodenal adenomatosis in familial adenomatous polyposis coli

Familial adenomatous polyposis coli (FAP) is an autosomal dominant genetic disorder caused by mutations of the APC gene on the long arm of chromosome 5. While multiple colorectal adenomas usually developing in early adolescence represent the most conspicuous phenotypic feature, the disease represents a generalized hyperproliferative disorder with various extracolonic manifestations. Duodenal cancer and desmoids are the leading causes of death in FAP patients after prophylactic colectomy. The prevalence of duodenal adenomatosis among FAP patients varies from 50% to greater than 90%, while only few patients (3-5%) develop duodenal cancer. Periampullary adenomas seem to carry a high risk of malignant transformation. The sensitivity of endoscopic procedures for early detection of severely dysplastic or malignant duodenal lesions is low, and the prognosis of duodenal cancer is poor. Thus the question arises whether it is possible to define a subgroup of high-risk patients for duodenal malignancy, and whether severe duodenal adenomatosis should lead to an aggressive prophylactic surgical approach. This contribution discusses the current literature and presents the experience of the Heidelberg Polyposis Register based on gastroduodenoscopy findings in 231 FAP patients. In 135 cases (58.4%) macroscopic duodenal adenomas were observed. The majority of patients displayed numerous lesions throughout the duodenum, while adenomas were restricted to the periampullary region in ten. Four patients suffered from duodenal cancer. Twenty-two required an endoscopic or operative intervention. Five were treated by laparotomy and duodenotomy, while ampullary excision was indicated in six cases. Eight patients underwent partial pancreaticoduodenectomy for severe duodenal adenomatosis.     

Hepatocyte nuclear factor-1 alpha gene inactivation: cosegregation between liver adenomatosis and diabetes phenotypes in two maturity-onset diabetes of the young (MODY)3 families

Heterozygous germline mutations of the hepatocyte nuclear factor (HNF)-1 alpha are associated with maturity-onset diabetes of the young (MODY)3. Recently, the biallelic inactivation of the HNF-1 alpha gene was reported in liver adenomas. We show the occurrence of liver adenomatosis in six MODY3-affected patients from two unrelated and large families. Liver adenomatosis was characterized by the presence of numerous adenomas within a normal hepatic parenchyma. The HNF-1 alpha hot-spot germline mutation P291fs was identified in the two probands and in 16 relatives from the two families. The six patients affected by liver adenomatosis and diabetes exhibited the mutation. The analysis of liver-cell tumors from two affected patients evidenced the biallelic inactivation of HNF-1 alpha. The familial screening confirmed the clinical heterogeneity of the liver phenotype, from silent liver adenomatosis to fatal hemorrhage. These observations warrant the systematic screening for liver adenomatosis in MODY3 families to prevent its potentially deadly complications. Moreover, such screening may help to determine if a particular mutational spectrum of HNF-1 alpha is associated with liver adenomatosis and to establish its prevalence in this frequent form of diabetes in the young adult.     

Biliary involvement in familial adenomatosis coli

Two patients with polypoid bile duct tumors associated with familial adenomatosis coli are presented. A jaundiced man, aged 63, had polypoid masses identified as adenocarcinoma in the common and hepatic ducts. The other patient, a 38-year-old woman undergoing surgery for duodenal adenomas, had a solitary benign adenoma of the distal common duct in addition to duodenal adenocarcinoma. Only one earlier report of bile duct polyps could be found in association with familial adenomatosis. These cases support the view that familial gastrointestinal adenomatosis is a systemic disease which is not restricted to the colon and rectum.     

Liver transplantation for benign liver tumors

Benign liver tumors are common lesions that are usually asymptomatic and are often found incidentally due to recent advances in imaging techniques and their widespread use. Although most of these tumors can be managed conservatively or treated by surgical resection, liver transplantation(LT) is the only treatment option in selected patients. LT is usually indicated in patients that present with life-threatening complications, when the lesions are diffuse in the hepatic parenchyma or when malignant transformation cannot be ruled out. However, due to the significant postoperative morbidity of the procedure, scarcity of available donor liver grafts, and the benign course of the disease, the indications for LT are still not standardized. Hepatic adenoma and adenomatosis, hepatic hemangioma, and hepatic epithelioid hemangioendothelioma are among the most common benign liver tumors treated by LT. This article reviews the role of LT in patients with benign liver tumors. The indications for LT and long-term outcomes of LT are presented.     

Gutartige Lebertumoren

The three clinically most relevant benign liver tumors and intrahepatic tumor-like lesions are (cavernous) hemangiomas, focal nodular hyperplasias (FNH) and hepatic adenomas (HCA). Most frequently, these lesions are detected as incidental findings in the context of abdominal ultrasound examinations and diagnosis can be accurately confirmed by dynamic, contrast-enhanced imaging (especially magnetic resonance imaging). Thus, liver histology is only required in rare, ambiguous cases. Importantly, the diagnosis of a benign liver lesion must be questioned and critically evaluated in all patients with pre-existing chronic liver disease, cirrhosis or known extrahepatic malignancies. Since the clinical course of hemangiomas and FNH is favorable and without complications, conservative strategies without long-term surveillance are usually recommended. However, in HCA occurrence of complications is directly related to the size of the lesion and comprises rupture or bleeding in approximately 30?% of the cases when size exceeds 5 cm. Depending on the proliferative capacity, gender, histological as well as molecular subtype (i.?e. β?HCA), risk of malignant transformation into hepatocellular carcinoma is also increased. Treatment strategies for HCAs primarily involve discontinuation of oral contraceptives, weight reduction and close monitoring of the lesions. If this approach is unsuccessful and lesions display progressive growth or cause persistent symptoms, resection should be performed. For HCA in men, resection is generally recommended regardless of the lesion size due to increased risk of malignant transformation.     

Unexpected discovery of 2 cases of hepatocyte nuclear factor 1α-mutated infracentimetric adenomatosis

We present 2 cases of hepatocyte nuclear factor 1α （HNF1α）-mutated adenomatosis, discovered for reasons unrelated to this disease, and identified using immunohistochemical methods. These new tools may further our understanding of the link between adenomas/adenomatosis subtypes and their complications, and their association with other abnormalities.     

Hepatic adenomatosis in an Hispanic patient. A case report and review of the literature

Liver adenomatosis (LA) is a rare disease originally defined by Flejou et al. in 1985 from a series of 13 cases. Only 57 cases have been reported in the literature, and all have been documented among Caucasian population. The aim of this study is to review and reappraise the characteristics of this rare liver disease, and to discuss the diagnosis and therapeutic options.LA is defined as the presence of >10 adenomas in an otherwise normal liver parenchyma. Neither female predominance nor a relation with estrogen/progesterone intake has been noted. Natural progression is poorly understood. We describe the clinical presentation, evolution, radiologic studies, histologic characteristics and therapeutic options in a 3rd generation Mexican woman with LA. We also include an updated review of the literature. The natural history and pathogenesis of LA are unclear. The risk of spontaneous hemorrhage or malignant transformation are a major concern. There is controversy regarding the optimal treatment for this disease; treatment options range from conservative medical therapy to surgical resection and even liver transplantation. LA is a rare disease, more common in women, and its outcome and evolution vary. Most often, conservative surgery is indicated. Liver transplantation is indicated only in highly symptomatic and aggressive forms of the disease.     

Recent concepts in non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease (NAFLD) is present in up to one-third of the general population and in the majority of patients with metabolic risk factors such as obesity and diabetes. Insulin resistance is a key pathogenic factor resulting in hepatic fat accumulation. Recent evidence demonstrates NAFLD in turn exacerbates hepatic insulin resistance and often precedes glucose intolerance. Once hepatic steatosis is established, other factors, including oxidative stress, mitochondrial dysfunction, gut-derived lipopolysaccharide and adipocytokines, may promote hepatocellular damage, inflammation and progressive liver disease. Confirmation of the diagnosis of NAFLD can usually be achieved by imaging studies, however, staging the disease requires a liver biopsy. NAFLD is associated with an increased risk of all-cause death, probably because of complications of insulin resistance such as vascular disease, as well as cirrhosis and hepatocellular carcinoma, which occur in a minority of patients. NAFLD is also now recognized to account for a substantial proportion of patients previously diagnosed with 'cryptogenic cirrhosis'. Diabetes, obesity and the necroinflammatory form of NAFLD known as non-alcoholic steatohepatitis, are risk factors for progressive liver disease. Current treatment relies on weight loss and exercise, although various insulin-sensitizing medications appear promising. Further research is needed to identify which patients will achieve the most benefit from therapy.     

Liver adenomatosis. An entity distinct from liver adenoma?

From 1979 to 1984, we followed the cases of 3 men (aged 13, 31, and 75 yr) and 2 women (aged 38 and 45 yr who had never used oral contraceptives) suffering from liver adenomatosis, an uncommon lesion consisting of numerous benign adenomas in an otherwise normal hepatic parenchyma. During the same period, we observed 20 cases of liver adenoma (one tumor in 18 patients and two tumors in 2 patients). From these cases and the review of previously reported cases of liver adenomatosis and series of liver adenoma, the following distinctive characteristics of these two benign conditions of the liver can be outlined: liver adenomatosis affects men and women, whereas liver adenoma predominantly affects women; liver adenomatosis is unrelated, whereas liver adenoma is closely related, to oral contraceptive use; increases in serum alkaline phosphatase and gamma-glutamyl transpeptidase are common in liver adenomatosis, but are uncommon in liver adenoma.     

Perioperative management of benign hepatic tumors in patients with glycogen storage disease type Ia

Glycogen storage disease type Ia (GSD-Ia; von Gierke disease) is an inherited disorder caused by glucose-6-phosphatase deficiency, and there have been some reports of hepatic tumors in patients with this disease. We report two patients with benign hepatic tumors with GSD-Ia. One is a 19-year-old man who underwent segmentectomy 4 for a focal nodular hyperplasia, and the other is a 31-year-old woman who underwent segmentectomies 3, 5, and 6 for hepatic adenomas. Two significant perioperative complications, resulting from the carbohydrate metabolic disorders, hypoglycemia and metabolic acidosis, occurred in both patients. We managed the metabolic complications successfully by administering a sufficient volume of glucose intravenously. Close perioperative monitoring of blood glucose and lactate concentrations is essential in the perioperative management of patients with GSD-Ia. The intravenous administration of glucose, starting with a smaller dose and then increasing the dose, is adequate management for lactic acidosis with or without hypoglycemia during the perioperative period.     

Successful photodynamic therapy for biliary papillomatosis： A case report

Papillomatosis of the bile duct is a rare disease with a high risk of malignant transformation. Therapeutical options include partial hepatectomy and liver trans- plantation. A previously healthy 65-years old male developed jaundice and right upper abdominal quadrant pain in 1996. A villous adenoma of the distal bile duct was diagnosed. A Whipple procedure was performed. In 2002 the patient turned symptomatic again. An- other adenoma was found in the right hepatic duct resulting in a right hepatectomy. Two years later the patient again developed cholestasis. Alter drainage of the left hepatic duct with a percutaneous transhepatic cholangial drainage （PTCD） catheter, a recurrent biliary adenomatosis was diagnosed by cholangioscopy. As there was no surgical option left, the patient received photodynamic therapy （PDT） for the recurrent biliary papillomatosis. Three mo alter he received further photodynamic therapies, the bile duct epithelium appeared normal and the patient had no signs of adenomatosis, both macroscopically and histologically. The follow-up cholangioscopy in late 2005 revealed only a small papil-loma without the need for intervention. In early 2006, the patient died of multi organ failure without signs of extrahepatic cholestasis or cholangitis at the age of 75, 10 years after the diagnosis of biliary papillomatosis was established. The patient exceeded the average life expectancy of patients with biliary papillomatosis by far. Thus, PDT might be a sufficient therapeutic option for recurrent papillomatosis patients with no significant side effects.     

Liver adenomatosis: re‐evaluation of aetiology and management

Background: Liver adenomatosis (LA) is a rare benign parenchymal liver disease with unknown aetiology. In recent reports, LA has been associated with hepatic steatosis, with potential implications for the management of this disease. The aim of this study was to determine prognosis and optimal management of patients with LA. Methods: Clinical presentation, diagnostic studies and management of patients with LA were analysed in our centre. Furthermore, a Medline search of all published case reports and series of LA patients was performed. Results: Ninety‐four patients with LA have been reported in the literature. Fifty‐two per cent of females had a history of oral contraceptive use. Eighteen per cent of patients had steatosis in nontumoral tissue. In our own series, five of six patients had histologically confirmed steatosis. Forty‐three per cent of patients presented with acute pain, of whom 46% had a haemorrhagical complication, in contrast to 2% of nonsymptomatic patients. Tumours <5 cm tended to increase in size during follow‐up and only in four patients tumour regression was observed. Conclusion: Liver adenomatosis is a progressive, benign parenchymal disease mainly occurring in females. There is a potential link with hepatic steatosis with implications for the management of patients with LA. Noninvasive diagnosis is difficult because of the variety of tumoral and nontumoral components. Management should primarily be conservative.