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.     

Intrahepatic rupture of a caudate lobe adenoma in liver adenomatosis

Hepatic adenomatosis is a rare benign disease that is more common in young and middle-aged women who are non-steroid dependent; it is usually symptomatic, progressive, and susceptible to hemorrhagic complications. Malignant transformation within adenomas is rare. The management of hepatic adenomatosis remains difficult due to the absence of predictive signs of complications, other than the size of the adenomas. Resective surgery is usually indicated, but liver transplantation could be an indication in highly symptomatic or progressive forms of the disease and represents the treatment of choice in cases of malignant transformation. We report a case of intrahepatic rupture of a caudate lobe adenoma which occurred in an adolescent with hepatic adenomatosis; we also present a brief review of the literature.     

Liver cell adenoma: A case report and a review of 30 cases reported in Japan

A 38 year old man with a huge liver cell adenoma extending to the pelvis was treated by left lateral lobectomy. The tumour, which was well-encapsulated by fibrous membrane, was 18 × 19 × 6 cm in size and weighed 1500g. Histopathologically, this tumour was composed of well-differentiated liver cell trabeculae and a small number of pseudobiliary ducts, but no Glisson sheaths. For the past 4 years 8 months he has remained asymptomatic. Thirty cases of liver cell adenoma found in Japanese literature are reviewed here and possible pathogenic implications are discussed.     

Liver cell adenoma showing sequential alteration of radiological findings suggestive of well-differentiated hepatocellular carcinoma

A liver tumor 35 mm in diameter was found incidentally in a 40-year-old woman who had no history of liver diseases or the use of oral contraceptives. Radiological diagnostics showed the typical findings of liver cell adenoma (LCA). Dynamic computed tomography revealed that the tumor showed a homogenous enhancement in the arterial phase and almost the same enhancement as the surrounding liver parenchyma in the delayed phase. The tumor was found to contain fat on magnetic resonance imaging. A benign fat containing liver tumor was suggested. However, radiological findings altered, which caused us to suspect that a welldifferentiated hepatocellular carcinoma (HCC) containing fat was becoming dedifferentiated. Partial hepatectomy was performed and the pathological findings showed the typical findings of LCA. This case was an extremely rare LCA, which had no background of risk for LCA and developed the sequential alteration of the radiological findings to suspect well-differentiated HCC.     

Liver cell adenoma in a young man with elevated serum PIVKA-II level

A 21-year-old man was referred to our hospital because of a liver mass lesion detected by abdominal ultrasonography. He had received no hormonal treatment. Physical examinations revealed no abnormalities, and laboratory data, including hepatic function test results, were within normal ranges, with the exception of elevated levels of serum protein induced by vitamin K absence or antagonist (PIVKA)-II (2.2 AU/ml). Abdominal ultrasonography revealed a hyperechoic mass lesion measuring 10×10 cm, with hypoechoic areas located in the right posterior segment of the liver. A low-density area and a hypervascular area were detected in the right posterior segment of the liver by computed tomography and celiac angiography, respectively. As hepatocellular carcinoma could not be completely excluded, the tumor was resected. The tissue consisted of sheets of tumor cells with eosinophilic cytoplasm and round nuclei showing a thin trabecular pattern, and these histological findings indicated liver cell adenoma. After resection of the tumor, serum PIVKA-II returned to the normal level.     

Benign myxoid hepatocellular tumor: a variant of liver cell adenoma

Abstract: A case of myxoid hepatocellular adenoma in the non-cirrhotic liver of a 26-year-old man is described. Grossly, the tumor was well circumscribed, rounded and measured 16 cm in diameter. Histologically, the tumor was characterized by nests and strands of polygonal cells embedded in a myxoid extracellular matrix. Electron microscopy confirmed the hepatocellular nature of the neoplastic cells. The patient is alive and well, without evidence of disease, 2 years after a local excision.     

Gardner's syndrome associated with periampullary carcinoma,duodenal and gastric adenomatosis

A 48-year-old man with Gardner's syndrome, who had abdominoperineal resection for rectal carcinoma in 1962, was found to have an ulcerating growth of the duodenum, and pancreaticoduodenectomy was performed in 1979. Histologic examination by complete step-serial sectioning disclosed a well-differentiated adenocarcinoma with adenomatous remnants, a large adenoma with focal carcinoma, 256 adenomas of the duodenum, and 91 adenomas of the gastric antrum. The world medical literature was reviewed, and 29 cases of periampullary carcinoma and 12 cases of gastric carcinoma complicating familial polyposis coli or Gardner's syndrome were analyzed.     

Liver cell adenoma： A case report with clonal analysis and literature review

We report a case of liver cell adenoma (LCA) in a 33-year-old female patient with special respect to its clonality status, pathogenic factors and differential diagnosis. The case was examined by histopathology, immunohistochemistry and a clonality assay based on X-chromosomal inactivation mosaicism in female somatic tissues and polymorphism at androgen receptor focus. The clinicopathological features of the reported cases from China and other countries were compared. The lesion was spherical, sizing 2 cm in its maximal dimension. Histologically, it was composed of cells arranged in cords, most of which were two-cell-thick and separated by sinusoids. Focal fatty change and excessive glycogen storage were observed. The tumor cells were round or polygonal in shape, resembling the surrounding parenchymal cells. Mitosis was not found. No portal tract, central vein or ductule was found within the lesion. The tumor tissue showed a positive reaction for cytokeratin (CK) 18, but not for CK19, vimentin, estrogen and progesterone receptors. Monoclonality was demonstrated for the lesion, confirming the diagnosis of an LCA. Clonality analysis is helpful for its distinction from focal nodular hyperplasia.     

Liver transplantation for recurrent hepatic adenoma

Hepatic adenoma (HA) is a rare indication for liver transplantation (LTx). So far 20 cases of LTx for HA are reported in PubMed. In rare cases HA presents as multiple hepatic adenomas or recurrent adenoma after initial liver resection and in such cases LTx is the only potential cure and prevents the risk of bleeding or cancer transformation into hepatocellular carcinoma. We report the case of a 56 years old lady who underwent a left hepatectomy for giant adenoma in 2005 and resection of segment Ⅴ-Ⅵ for recurrence of liver adenoma in 2007. She developed a second recurrence of HA with 3 new lesions in the right liver in 2008. The patient underwent LTx. After 3 years the patient is alive with no evidence of disease. LTx is indicated in patients with HA in which resection is not technically feasible.     

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.     

肝腺瘤组织肝脏功能水平的研究

目的初步探索肝腺瘤组织细胞所具备的合成、代谢、解毒等肝脏功能活性水平,为其能否成为人工肝新型细胞材料来源提供依据。方法收集近三年来南方医院手术切除并经病理证实为肝腺瘤的组织标本3例,HE染色后电子显微镜下观察其细胞形态及组织结构,并通过糖原染色、免疫组化及qRT-PCR等方法检测肝细胞合成、代谢和转化等功能相关基因的表达情况,同时与正常肝组织细胞的功能水平进行对比。结果肝腺瘤细胞形态与正常肝细胞相似,细胞核无明显异型性,qRT-PCR检测发现肝腺瘤各肝功能相关基因的表达均与正常肝组织细胞相当,部分指标甚至高于正常肝组织细胞。免疫组化检测发现肝腺瘤和正常肝组织中ALB、P450和CK18均有较强表达,AFP除一例肝腺瘤组织有部分表达外余2例和全部正常组织均无表达。结论肝腺瘤细胞有望在解决生物人工肝细胞来源缺乏问题上产生重大突破,有必要进一步开展肝腺瘤的建株研究。     

Hepatocellular adenoma: An update

Hepatocellular adenomas (HCA) are rare benign liver tumors. Recent technological advancements have helped in the early identification of such lesions. However, precise diagnosis of hepatocellular incidentalomas remains challenging. Studies at the molecular level have provided new insights into the genetics and pathophysiology of these lesions. These in turn have raised questions over their existing management modalities. However, the rarity of the tumor still restricts the quality of evidence available for current recommendations and guidelines. This article provides a comprehensive review on the etiology, molecular biology, patho-physiology, clinical manifestations, and complications associated with HCA. It also elaborates on the genetic advancements, existing diagnostic tools and current guidelines for management for such lesions.     

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.     

Hepatocellular adenoma: an excellent indication for laparoscopic liver resection

Objectives

Laparoscopic resection for benign liver disease has gained wide acceptance in recent years and hepatocellular adenoma (HA) seems to be an appropriate indication. This study aimed to discuss diagnosis and treatment strategies, and to assess the feasibility, safety and outcomes of pure laparoscopic liver resection (LLR) in a large series of patients with HA.

Methods

Of 88 patients who underwent pure LLR, 31 were identified as having HA. Diagnosis was based on radiological evaluation and resections were performed for lesions measuring >5.0 cm.

Results

The sample included 29 female and two male patients. Their mean age was 33.2 years. A total of 27 patients had a single lesion, one patient had two and one had four lesions. The two remaining patients had liver adenomatosis. Mean tumour size was 7.5 cm. Three right hepatectomies, 17 left lateral sectionectomies and 11 wedge resections or segmentectomies were performed. There was no need for blood transfusion or conversion to open surgery. Postoperative complications occurred in two patients. Mean hospital stay was 3.8 days.

Conclusions

Hepatocellular adenoma should be regarded as an excellent indication for pure LLR. Pure LLR is safe and feasible and should be considered the standard of care for the treatment of HA when performed by surgeons with experience in liver and laparoscopic surgery.     

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.     

肝腺瘤的磁共振成像诊断

研究及阐述肝腺瘤的MRI表现与诊断 ,以提高MRI对肝腺瘤的诊断能力。对我院从 1991～ 2 0 0 3年在受检 4 138例肝脏病例中 ,共收集到的 2例材料齐全的肝腺瘤病例 ,并经MRI、CT、US检查及手术和病理证实。 1例肝腺瘤MRI表现为T1WI上高信号 ,T2 WI上稍高信号 ,SPIR(脂肪抑制像 )表现为稍低信号 ,FFE(快速梯度回波 )表现为稍低信号。另 1例肝细胞腺瘤在T1WI为稍低信号 ,T2 WI稍高信号 ,其内见斑片状高信号 ,SPIR表现与T2 WI基本相同。MRI对肝腺瘤的术前诊断具有一定的临床价值     

Diagnosis of hepatocellular adenoma in men before onset of diabetes in HNF1A‐MODY: Watch out for winkers

Hepatocyte nuclear factor 1A (HNF1A) maturity‐onset diabetes of the young (MODY) is a monogenetic, autosomal dominantly inherited form of diabetes. HNF1A‐MODY is associated with HNF1A‐inactivated hepatocellular adenoma (H‐HCA) formation. Hepatocellular adenoma (HCA) are benign liver tumours and related complications are rare but serious: hepatic haemorrhage and malignant transformation. Guidelines recommend resection of all HCA in men and do not take any co‐occurring metabolic disorders into account. We report a family with HCA preceding diabetes mellitus. Male index patient presented with numerous, irresectable HCA. After initial diagnostic and aetiologic uncertainty HNF1A germline mutation c.815G>A (p.Arg272His) was confirmed 8 years later. No HCA‐related complications occurred. His diabetic mother was diagnosed with HCA after severe hepatic haemorrhage years before. HNF1A‐MODY should be considered in (non‐)diabetic (male) patients with H‐HCA. We advocate liver biopsy and, if necessary, genetic analysis to precede any intervention for HCA in males and screening for HCA in HNF1A‐MODY patients.     

Two-stage liver transplant for ruptured hepatic adenoma:A case report

BACKGROUND Only one case of liver transplantation for hepatic adenoma has previously been reported for patients with rupture and uncontrolled hemorrhage.We present the case of a massive ruptured hepatic adenoma with persistent hemorrhagic shock and toxic liver syndrome which resulted in a two-stage liver transplantation.This is the first case of a two-stage liver transplantation performed for a ruptured hepatic adenoma.CASE SUMMARY A 23 years old African American female with a history of pre-diabetes and oral contraceptive presented to an outside facility complaining of right-sided chest pain and emesis for one day.She was found to be in hemorrhagic shock due to a massive ruptured hepatic hepatic adenoma.She underwent repeated embolizations with interventional radiology with ongoing hemorrhage and the development of renal failure,hepatic failure,and hemodynamic instability,known as toxic liver syndrome.In the setting of uncontrolled hemorrhage and toxic liver syndrome,a hepatectomy with porto-caval anastomosis was performed with liver transplantation 15 h later.She tolerated the anhepatic stage well,and has done well over one year later.CONCLUSION When toxic liver syndrome is recognized,liver transplantation with or withouthepatectomy should be considered before the patient becomes unstable.     

Pyogenic liver abscess associated with large colonic tubulovillous adenoma

Pyogenic liver abscesses usually occur in associationwith a variety of diseases.Rarely,liver abscess has beenreported as the presenting manifestation of colonic tubu-lovillous adenoma.We report two cases of pyogenic liverabscess without hepatobiliary disease or other obviousetiologies except that one had a history of diabetesmellitus(DM).The pathogen in the patient with DM wasKlebsiella pneurnonia(KP).In both of the patients,ileusdeveloped about two to three weeks after the diagnosisof liver abscess.Colonoscopy revealed large polypoidtumors with pathological findings of tubulovillousadenoma in both cases.Two lessons were learned fromthese two cases:(1)an underlying cause should beaggressively investigated in patients with cryptogenicliver abscess;(2)DM could be one of the etiologies butnot necessarily the only cause of KP liver abscess.