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Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1975 to 1989, 51 patients presenting with hepatocellular carcinoma complicating genetic haemochromatosis were managed in our institution. Twenty-one patients underwent a laparotomy. Laparotomy was limited to surgical exploration or palliation in 8 patients in whom the tumour was deemed unresectable. Thirteen patients underwent either a partial hepatectomy (11 patients) or a total hepatectomy and liver transplantation (2 patients). Actuarial survival at 1 and 3 years following partial hepatectomy was 56% and 40% respectively. There was one hospital death in the resection group and in the transplant group. Only 3 patients have remained free of tumour recurrence after a mean follow-up of 18 months. Common clinical and histological features for patients with this condition included masculine gender, age 50 years or above, 10 or more years of history of diagnosed genetic haemochromatosis, high alcohol intake, and grade III or IV hepatic fibrosis.
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PMID:Surgical management of hepatocellular carcinoma in genetic haemochromatosis. 784 88

Cross-sectional imaging is playing an increasing role in diagnosis of diffuse liver diseases because it clarifies, in many cases, the overlap in clinical and laboratory manifestations often present in diffuse hepatic processes and thus may eliminate the need for a biopsy. Advances in cross-sectional imaging, particularly in magnetic resonance (MR) imaging, enable further characterization of hepatic parenchymal and architectural changes, allowing closer correlation with underlying pathologic changes. Advanced imaging techniques can be used to characterize a variety of metabolic, vascular, toxic, infectious, and neoplastic diffuse liver diseases. These include more common entities such as cirrhosis, Budd-Chiari syndrome, hemochromatosis, Wilson disease, fatty change, and diffuse neoplastic disease (hepatocellular carcinoma, metastasis, and lymphoma) and uncommon entities such as schistosomiasis, sarcoidosis, and amyloidosis. Correlation of computed tomographic and MR imaging findings with underlying pathologic features is helpful in understanding the gamut of diffuse diseases of the liver.
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PMID:Diffuse disease of the liver: radiologic-pathologic correlation. 785 42

Sublobular nodules of hepatocytes free of iron or exhibiting much less iron than the surrounding parenchyma, referred to in this study as iron-free-foci, are frequently found in the livers of patients with genetic hemochromatosis complicated by hepatocellular carcinoma. To test the hypothesis that such nodules are preneoplastic lesions, iron-free foci were sought in the initial liver biopsy specimens of 185 patients with untreated and uncomplicated genetic hemochromatosis. Iron-free foci were found in 14 (7.6%) patients, all men, aged from 38 to 76 yr, with heavy iron overload and with fibrosis or cirrhosis. Twelve patients with iron-free foci were followed for 0.9 to 15 yr (7 +/- 6 yr). In six (50%), HCC developed, compared with 2 (8%) from a control group consisting of 24 patients without IFF matched according to age, sex, degree of fibrosis, liver iron amount and follow-up duration. The mean number of iron-free foci per iron-free foci-positive specimen was 3.2 +/- 2.1. Ten patients had dysplastic aspects in their iron-free foci, and four had intrahepatocytic iron-positive inclusions at the periphery of iron-free foci. Proliferative cell nuclear antigen was positive in 75% of iron-free foci and in 24% +/- 21% of hepatocyte nuclei in iron-free foci. This study clearly demonstrates that iron-free foci are proliferative lesions and strongly suggests that such nodules are preneoplastic foci. Therefore the finding of IFF in the initial liver biopsy specimen from a patient with genetic hemochromatosis should lead to regular screening for hepatocellular carcinoma.
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PMID:Preneoplastic significance of hepatic iron-free foci in genetic hemochromatosis: a study of 185 patients. 790 16

Hereditary hemochromatosis is a prevalent inherited disorder with an estimated frequency of homozygosity of 0.2 to 0.45% in Caucasians. The disease is characterized by progressive iron overload until a massive accumulation of body iron occurs. Undetected, the disorder eventually can produce either cirrhosis, diabetes mellitus, cardiac disease, arthritis, or hepatocellular carcinoma or a combination of these manifestations. Early diagnosis and treatment prevents organ damage and normalizes life expectancy. Screening studies to detect hemochromatosis are most effectively accomplished by measurement of the serum iron and total iron binding capacity. Treatment is most effectively performed by frequent phlebotomy until body stores are empty and then 3 to 4 times yearly for life. The basic defect of hemochromatosis appears to increase iron absorption, decrease iron excretion, and produce preferential deposit of iron in hepatic parenchymal cells rather than Kupffer cells. The genetic abnormality of hemochromatosis is located on chromosome 6 in close association with the gene for HLA antigens. Recent speculation postulates that tumor necrosis factor may be involved in the etiology of this disease because of its location on chromosome 6 and its effect upon iron transport.
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PMID:Hereditary hemochromatosis: a prevalent disorder of iron metabolism with an elusive etiology. 794 87

To identify factors that might be useful as prognostic indexes for the risk of hepatocellular carcinoma in Italian patients with genetic hemochromatosis, 152 homozygotes were studied prospectively for 1 to 229 mo. Factors that were considered in estimating the risk of developing hepatocellular carcinoma were age, sex, cirrhosis (Child class), HBsAg, antibodies to HBsAg, antibodies to HBcAg, hepatitis C antibodies, alcohol abuse and the amount of iron removed during therapeutic phlebotomy to produce iron depletion. At diagnosis, cirrhosis was present in 97 patients and absent in 55. During follow-up, hepatocellular carcinoma developed in 28 of the 97 patients with cirrhosis but in none of those without. Among patients with cirrhosis, the cumulative probability of being free of hepatocellular carcinoma at 10 yr was 70%. For patients with and without HBsAg the probabilities of being free of liver cancer at 10 yr were, respectively, 54% and 75%; for those with and without history of alcoholism, 58% and 78%; and for those younger and older than 55 yr, 90% and 54%. In patients with cirrhosis, multivariate analysis using proportional-hazards (Cox) regression found that the only factors contributing significantly to the estimation of a prognostic index were age, presence of HBsAg and alcohol abuse. Age over 55 yr increased the relative risk of hepatocellular carcinoma 13.3-fold (p < 0.001), the presence of HBsAg increased it 4.9-fold (p < 0.02) and alcohol abuse increased it 2.3-fold (p < 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prognostic factors for hepatocellular carcinoma in genetic hemochromatosis. 798 40

We describe the establishment and characterization of a novel hepatoma cell line. This cell line, designated RBHF-1, was established from a hepatocellular carcinoma of a 67-yr-old man with a history of genetic hemochromatosis. At this writing, the cells have been maintained in RPMI-1640 tissue-culture medium and fetal calf serum without any additional supplements for 30 mo. The cells form colonies on soft agar and are not tumorigenic in nude mice. The cell line is polymorphic and displays characteristics of mature hepatocytes by synthesizing albumin, alpha 2-macroglobulin, fibronectin and alpha-fetoprotein. Cytogenetic analysis shows multiple chromosomal aberrations, with a consistent deletion in the long arm and deletions or rearrangements in the short arm of chromosome 1. There is no evidence for hepatitis B or hepatitis C virus infection of the cell line. The cells contain no detectable intracellular iron after staining with Perls' stain. Unlike other hepatoma cell lines, there is no detectable binding of epidermal growth factor to RBHF-1 cells. This is the first cell line to be established from a patient with hemochromatosis, and it provides a potentially important model for the study of hepatocyte transformation in association with iron overload.
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PMID:Establishment of a cell line from a hepatocellular carcinoma from a patient with hemochromatosis. 802 Sep 7

During the period 1986-93 22 patients were diagnosed as having primary hemochromatosis. Only 11 of them had elevated aminotransferases. Transferrin saturation was higher > 63% in 17 (77%) and serum-ferritin was higher in all the patients. (257 mumol/l to 6,500 mumol/l). A percutaneous liver biopsy was performed in 20 patients, all of whom showed a characteristic grading from 2 + to 4+ using Perls' stain. Two males had cirrhosis with simultaneous hepatocellular carcinoma, and another two had cirrhosis. One patient had diabetes mellitus type I. We conclude that fasting serum-iron and transferrin should be determined in all subjects over 40 years of age and in patients with chronic elevation of liver enzymes. If transferrin saturation is higher than 50% in females and 60% in males, serum ferritin should be determined. A percutaneous liver biopsy should be performed if both values are higher than normal. Screening of siblings is important because of the autosomal recessive pattern of inheritance.
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PMID:[Clinical experience with early hemochromatosis]. 807 82

Haemochromatosis is an inherited disorder of iron metabolism characterized by a general iron over loading. Without diagnosis and early treatment, it is a serous and potentially fatal disease by cardiac failure or hepatocellular carcinoma in particular. Gene prevalence was estimated at 0.06 in Brittany, so that haemochromatosis may be the most common genetic disease in this area. The biochemical defect of the disease is unknown; only one fact is well established: the iron absorption through duodenal mucosa is excessive. However, we don't know if it is a primary event. The gene is also unknown but in 1975 it was located on the short arm of chromosome 6, closely linked to the HLA class I region, less than 1 cM from HLA-A. None of the genes coding for the known iron proteins could be the haemochromatosis gene because of their chromosomal localization. In order to locate this gene with precision, we have used a reverse genetic approach now called positional cloning. Characterization of new polymorphic markers and linkage disequilibrium analysis have led us to locate the gene within a 350 kb region around HLA-A. We have then searched for all the structural genes in this region. Seven new genes have been so identified and located with precision. A structural analysis of these genes was undertaken to find an eventual abnormality in patients.
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PMID:[Molecular genetics of hemochromatosis]. 811 56

Twelve years after receiving radiation therapy with thorium X (280 microCi) for long-standing Bechterew's disease (ankylosing spondylitis) a 52-year-old man was found, by ultrasonography and computed tomography, to have a round mass, 11 x 12 cm, in the left lobe of the liver. Laparoscopy discovered coarse, discoloured nodes on the surface of the right and left lobes of the liver which histologically showed hepatocellular carcinoma. There were no known risk factor for liver carcinoma (like cirrhosis, positive hepatitis B serology, alcohol abuse, haemochromatosis or alpha 1-antitrypsin deficiency). As exploratory laparotomy found the tumour to be inoperable, 15 chemotherapeutic embolizations were performed. An abdominal wall metastasis was resected after 17 months. At the time of this report, 20 months after the diagnosis was first made, the patient is in a poor general condition. Internal radiotherapy with thorium X was used, all else having failed, in the treatment of severe ankylosing spondylitis. Although it is not possible to prove a direct causal relationship between the thorium X radiation and development of a liver carcinoma, the coincidence is remarkable.
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PMID:[Hepatocellular carcinoma following intravenous thorium X therapy]. 818 11

Haemochromatosis is an inherited disorder of iron metabolism characterized by a general iron over loading. Without diagnosis and early treatment, it is a serious and potentially fatal disease by cardiac failure or hepatocellular carcinoma in particular. Gene prevalence was estimated at 0.06 in Brittany, so that haemochromatosis may be the most common genetic disease in this area. The biochemical defect of the disease is unknown; only one fact is well established: the iron absorption through duodenal mucosa is excessive. However we don't know if it is a primary event. The gene is also unknown but in 1975 it was located on the short arm of chromosome 6, closely linked to the HLA class I region, less than 1 cM from HLA-A. None of the genes coding for the known iron proteins could be the haemochromatosis gene because of their chromosomal localization. In order to locate this gene with precision, we have used a reverse genetic approach now called positional cloning. Characterization of new polymorphic markers and linkage disequilibrium analysis, have led us to locate the gene within a 350 kb region around HLA-A. We have then searched for all the structural genes in this region. Seven new genes have been so identified and located with precision. A structural analysis of these genes was undertaken to find an eventual abnormality in patients.
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PMID:[Molecular genetics of hemochromatosis]. 835 73


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