Hepatocellular carcinoma in a noncirrhotic patient with hereditary hemochromatosis

We report the case of a 66-year-old man with genetic hemochromatosis who was found to have hepatocellular carcinoma (HCC) in the absence of cirrhosis. This is a rare and life-threatening complication of noncirrhotic hemochromatosis that has been described only 10 times in the English literature. In addition to presenting our case, we also cite some other potential causes of HCC in noncirrhotic patients that should be clinically considered. Although the incidence of HCC in noncirrhotic hemochromatosis patients is not sufficiently high to warrant routine screening, physicians should be aware that this fatal complication may rarely occur.     

Hepatocellular carcinoma without cirrhosis in a patient with nonalcoholic steatohepatitis

All cases of hepatocellular carcinoma reported thus for in patients with nonalcoholic steatohepatitis have occurred on preexisting cirrhosis. We report the case of a 68-Year-old male patient with nonalcoholic steatohepatitis who developed hepatocellular carcinoma in the absence of cirrhosis. This observation suggests a possible relationship between nonalcoholic steatohepatitis and/or excess body weight, and hepatocellular carcinoma independent of cirrhosis. Further epidemiological studies are needed to evaluate the incidence of this association.     

Hepatocellular carcinoma associated with hereditary hemochromatosis occurring in non-cirrhotic liver

The occurrence of primary hepatocellular carcinoma (HCC) in patients with hereditary hemochromatosis (HH) is well known. Thereby, the development of liver cirrhosis seems to be a prerequisite. Whether or not a hepatic iron overload in the context of hereditary hemochromatosis is an independent risk factor for HCC remains unclear. To date there are only a few reports about HCC arising in non-cirrhotic livers in the presence of HH. We report the case of a 64-year-old man who presented to our outpatient clinic with HCC. Liver cirrhosis could be excluded. Detailed exploration of the patient's history revealed that he had been treated by venesection for about 10 years up to 15 years ago. Subsequent investigations showed an elevated serum ferritin and transferrin saturation. The diagnosis of HH was confirmed by genetic testing, with homozygosity for the Cys282Tyr mutation. The patient received palliative chemotherapy and finally died 15 months after initial diagnosis of HCC.     

Hepatocellular carcinoma in a patient with precirrhotic primary biliary cirrhosis

Hepatocellular carcinoma is generally associated with long-standing chronic liver disease of diverse etiology, most commonly HBsAg-positive chronic active hepatitis, hemochromatosis, or alcoholic liver disease. Patients with primary biliary cirrhosis have only rarely developed a subsequent hepatocellular carcinoma. We report such a patient, a 77-year-old woman with an early, precirrhotic stage of primary biliary cirrhosis who developed a hepatoma.     

原发性血色病一例报道

1病例资料患者男,64岁,公路沥青工人。因"间断乏力,头晕5年余,加重伴腹胀,尿少4个月"以"肝硬化"收入院。5年前因间断乏力,头晕,诊断为继发性再障。4个月前因腹胀,尿少(24 h尿量为700～800 ml),空腹     

Hepatocellular carcinoma in non-alcoholic steatohepatitis without cirrhosis

Cirrhosis is an emerging major cause of the development of hepatocellular carcinoma (HCC), but in non-alcoholic fatty liver disease (NAFLD), up to 50% of patients with HCC had no clinical or histological evidence of cirrhosis. It is currently challenging to propose general recommendations for screening patients with NAFLD without cirrhosis, and each patient should be evaluated on a case-by-case basis based on the profile of specific risk factors identified. For HCC screening in NAFLD, a valid precision-based screening is needed. Currently, when evaluating this population of patients, the use of non-invasive methods can guide the selection of those who should undergo a screening and surveillance program. Hence, the objective of the present study is to review the epidemiology, the pathophysiology, the histopathological aspects, the current recommendations, and novel perspectives in the surveillance of non-cirrhotic NAFLD-related HCC.     

Hepatocellular carcinoma without cirrhosis in Japanese patients

Hepatocellular carcinoma is closely associated with cirrhosis, but it also develops, although much less frequently, in a noncirrhotic liver. It is suspected, without supporting evidence, that hepatocellular carcinoma has a different etiology when associated and not associated with chronic liver disease. In this study, 66 noncirrhotic cases found among 618 autopsies for hepatocellular carcinoma (10.7%) were analyzed retrospectively. The noncirrhotic liver was histologically unremarkable in 3 cases and in the histologically evaluable 56 cases it had fibrosis of varying degrees or mild cellular infiltrate, or both, in the portal tract. There was one liver that had portal venous changes compatible with those in idiopathic portal hypertension (Banti's syndrome). In these noncirrhotic livers, the parenchymal cells were generally unremarkable except for liver cell dysplasia that was seen in 26.8%. Serum hepatitis B surface antigen was positive in only 7.4% in contrast to 26.6% in cirrhotic cases. Three histologically unremarkable cases had no clinical or histologic evidence of chronic liver disease; two involved painter-plasterers and one a farmer. The liver weight in these cases ranged from 4400 to 6180 g. In contrast, the average liver weight in cirrhotic cases was 1998 g. Noncirrhotic patients when compared with cirrhotic patients had better liver function tests and much less frequent varices. It was concluded that approximately 11% of hepatocellular carcinoma cases in Japan are noncirrhotic, the majority having some histologic changes in the portal tracts suggestive of past or ongoing chronic liver disease, and that there are rare cases that have no histologic changes in the liver.     

Hepatocellular carcinoma in patients without cirrhosis in Italy

BackgroundIn the Western world, hepatocellular carcinoma seldom develops in patients without cirrhosis, and reports describing the characteristics of non-cirrhotic patients with hepatocellular carcinoma are rather infrequent.MethodsWe evaluated the main clinical characteristics, treatment options, and survival of patients with hepatocellular carcinoma developed in non-cirrhotic liver among the 3027 consecutive cases of hepatocellular carcinoma accrued in the Italian Liver Cancer database during the last 20 years.ResultsWe identified 52 patients with hepatocellular carcinoma in non-cirrhotic livers (1.7% of all hepatocellular carcinomas), 42 with (80.8%) and 10 without (19.2%) chronic liver disease. In patients without chronic liver disease, median tumour diameter was greater compared to patients with chronic liver disease (7.8 versus 4.0 cm, P = 0.046). Curative treatment was feasible in 20 patients (38.5%). Median overall survival was 26 months and 5-year survival rate was 23.7%. Detection of hepatocellular carcinoma outside surveillance (P = 0.036), advanced hepatocellular carcinoma stage (P < 0.0001), and non-curative treatment (P = 0.007) were associated with worse prognosis, but tumour stage was the only independent predictor of survival.ConclusionsIn Italy, less than 2% of hepatocellular carcinomas develop in a non-cirrhotic liver, and almost never in a normal liver. These patients frequently present with advanced tumours, have low eligibility rates for curative treatment, and have a dismal prognosis despite their preserved liver function.     

Hepatocellular carcinoma in patients with chronic hepatitis C virus infection without cirrhosis

AIM:To investigate and characterise patients with chronic hepatitis C virus(HCV) infection presenting with hepatocellular carcinoma(HCC) in the absence of cirrhosis.METHODS:Patients with chronic hepatitis C infection without cirrhosis presenting with HCC over a 2-year period were identified.The clinical case notes,blood test results and histological specimens were reviewed to identify whether additional risk factors for the development of HCC were present.RESULTS:Six patients(five male,one female) with chro...     

Hepatocellular carcinoma with and without cirrhosis. A comparison in southern African blacks

Hepatocellular carcinoma and cirrhosis frequently coexist. In populations with a low incidence of hepatocellular carcinoma, the tumor often arises as a complication of long-standing symptomatic cirrhosis, which may be micronodular or macronodular and which is usually alcoholic in origin, and cirrhosis per se is the major etiologic association of the tumor. The relation between these two pathologic conditions in populations with a high incidence of hepatocellular carcinoma has not hitherto been analyzed. In this study the association was examined in 463 southern African black men with hepatocellular carcinoma. Cirrhosis, almost always macronodular and rarely showing features of alcholic toxicity, was present in 63.1% of the patients. No differences were found in the age structure, clinical features, hepatic function, serum alpha-fetoprotein concentrations, or hepatitis B virus status between patients with hepatocellular carcinoma with and without cirrhosis. Patients with cirrhosis survived slightly longer, but the difference was not biologically significant. It is concluded that the relation between hepatocellular carcinoma and cirrhosis in southern African blacks differs substantially from that in low incidence regions of the tumor.     

Hepatocellular carcinoma in young, mentally retarded HBsAg carriers without cirrhosis

In a Californian institution for the mentally retarded, surveillance by autopsy of all deaths (n = 138) identified three cases of hepatocellular carcinoma. Hepatocellular carcinoma cases occurred in young (mean age = 26 years) male carriers of HBsAg and were not associated with cirrhosis. They were of the nonfibrolamellar oncocytic type and were rapidly fatal. Hepatocellular carcinoma incidence in HBsAg carriers was estimated to be 246 times greater than United States males.     

Prognostic factors and survival in patients with hereditary hemochromatosis and cirrhosis.

OBJECTIVES: The survival of treated, noncirrhotic patients with hereditary hemochromatosis is similar to that of the general population. Less is known about the outcome of cirrhotic hereditary hemochromatosis patients. The present study evaluated the survival of patients with hereditary hemochromatosis and cirrhosis. METHODS: From an established hereditary hemochromatosis database, all cirrhotic patients diagnosed from January 1972 to August 2004 were identified. Factors associated with survival were determined using univariate and multivariate regression. Survival differences were assessed using the Kaplan-Meier life table method. RESULTS: Ninety-five patients were identified. Sixty patients had genetic testing, 52 patients (87%) were C282Y homozygotes. Median follow-up was 9.2 years (range 0 to 30 years). Nineteen patients (20%) developed hepatocellular carcinoma, one of whom was still living following transplantation. Cumulative survival for all patients was 88% at one year, 69% at five years and 56% at 20 years. Factors associated with death on multivariate analysis included advanced Child-Pugh score and hepatocellular carcinoma. Patients with hepatocellular carcinoma were older at the time of diagnosis of cirrhosis (mean age 61 and 54.6 years, respectively; P=0.03). The mean age at the time of diagnosis of hepatocellular carcinoma was 70 years (range 48 to 79 years). No other differences were found between the groups. CONCLUSIONS: Patients with hereditary hemochromatosis and cirrhosis are at significant risk of developing hepatocellular carcinoma. These patients are older when diagnosed with carcinoma and may have poorer survival following transplantation than patients with other causes of liver disease. Early diagnosis and treatment of hereditary hemochromatosis by preventing the development of cirrhosis may reduce the incidence of hepatocellular carcinoma in the future.     

Hepatocellular carcinoma development in cirrhosis

The development of hepatocellular carcinoma (HCC), the mechanisms of hepatocarcinogenesis, the prevention of HCC, and screening for HCC will be discussed. Cirrhosis has been considered as a pre-neoplastic condition for the development of HCC. The worldwide incidence of HCC differs according to different hepatitis viruses, and information is lacking. Hepatocarcinogenesis is a multistep process involving a number of different genetic alterations and is poorly understood. Interferon should help prevent the development of HCC in patients with chronic hepatitis C. Screening is the only practical approach for improving the management of HCC patients, as early detection increases the application of curative treatments. However, the cost-effectiveness of various screening strategies needs to be analysed.     

Hepatocellular carcinoma in a male patient with early stage (stage I) primary biliary cirrhosis

The true incidence of hepatocellular carcinoma (HCC) in patients with primary biliary cirrhosis (PBC) remains undetermined due to limited epidemiological studies and some conflicting results. Some studies indicated that in PBC, male gender, cirrhosis, hepatitis C virus (HCV) superinfection, and history of blood transfusion are associated with the development of HCC, and the occurrence of HCC in the early stage of PBC is rare. We present herein a 75-year-old male patient with stage I PBC who developed oropharyngeal squamous cell carcinoma, followed by HCC and duodenal adenocarcinoma without hepatitis B or C virus infection. While it could be argued that the concurrence of HCC and stage I-PBC in our patient was coincidental, patients with early stage PBC should be strictly followed up as cirrhotic patients with PBC by monitoring the serum concentration of tumor markers for HCC and appropriate imaging methods.