Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Porphyria cutanea tarda (PCT), the most common form of porphyria, may be one of the rare cutaneous manifestations of hepatic tumors, benign, malignant, or metastatic. The liver damage associated with PCT may predispose to the development of hepatocellular carcinoma. Our experience and a review of the literature suggest that in patients with PCT in whom the usual precipitating factors are absent, or in patients with PCT of long duration and an unexplained exacerbation, liver scan is indicated to rule out the presence of a hepatic tumor.
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PMID:Porphyria cutanea tarda. A rare cutaneous manifestation of hepatic tumors. 20 91

Thirty-eight patients with porphyria cutanea tarda (PCT) have been seen in the last 18 years. Five of these patients (13%) developed hepatocellular carcinoma (HCC) during follow-up. We analyzed the differences in clinical, laboratory and liver histology findings at presentation, between patients who developed HCC during follow-up (HCC-group, n = 5) and those who did not (PCT-group, n = 33). Of the clinical features the duration of skin-symptoms was longer in the HCC-group (mean: 10.4 +/- 1.1 years) than in the PCT-group (mean: 1.4 +/- 1 years) (p less than 0.001). No differences in routine laboratory findings were found. Although 11/38 (29%) patients had serologic evidence of a past hepatitis B virus infection and 7/38 (18%) patients had antibodies against hepatitis C virus, no differences in these parameters were found between the PCT-group and the HCC-group. In all 34 liver biopsies a variable degree of siderosis was found (PCT-group vs. HCC-group: NS). Only piecemeal necrosis (p less than 0.01) and advanced fibrosis or cirrhosis (p less than 0.001) were more common in liver biopsies in the HCC-group. In conclusion, factors related to an increased risk of HCC in PCT are: a) a long symptomatic period before start of treatment and b) the presence of chronic active hepatitis and/or advanced fibrosis or cirrhosis in liver biopsies.
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PMID:Hepatocellular carcinoma in porphyria cutanea tarda: frequency and factors related to its occurrence. 132 Jan 75

Iron is essential for life, but iron overload is toxic and potentially fatal. The liver is a major site of iron storage and is particularly susceptible to injury from iron overload, especially when (as in primary hemochromatosis) the iron accumulates in hepatocytes. Iron can be taken up by the liver in several forms and by several pathways including: (1) receptor-mediated endocytosis of diferric or monoferric transferrin or ferritin, (2) reduction and carrier-facilitated internalization of iron from transferrin without internalization of the protein moiety of transferrin, (3) electrogenic uptake of low molecular weight, non-protein bound forms of iron, and (4) uptake of heme from heme-albumin, heme-hemopexin, or hemoglobin-haptoglobin complexes. Normally, pathway 2 is probably the major one for uptake of iron by hepatocytes. Iron is stored in the liver in the cores of ferritin shells and as hemosiderin, an insoluble product derived from iron-rich ferritin. Iron in hepatocytes stimulates translation of ferritin mRNA and represses transcription of DNA for transferrin and transferrin receptors. The major pathologic effects of chronic hepatic iron overload are: (1) fibrosis and cirrhosis, (2) porphyria cutanea tarda, and (3) hepatocellular carcinoma. Although precise pathogenetic mechanisms remain unknown, iron probably produces these and other toxic effects by increasing oxidative stress and lysosomal lability. Vigorous efforts at diagnosis and treatment of iron overload are essential since the pathologic effects of iron are totally preventable by early vigorous iron removal and prevention of iron re-accumulation.
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PMID:Iron and the liver. 184 76

Alcoholic liver disease includes steatosis, alcoholic hepatitis and cirrhosis. Other liver diseases of genetic origin, but with a curious association with alcohol intake, are hemochromatosis and porphyria cutanea tarda. The attribution of chronic hepatitis to alcohol intake remains speculative, and the association may reflect hepatitis C infection. Hepatic injury attributed to alcohol includes the changes reported in the fetal alcohol syndrome. Steatosis, the characteristic consequence of excess alcohol intake, is usually macrovesicular and rarely microvesicular. Acute intrahepatic cholestasis, which in rare instances accompanies steatosis, must be distinguished from other causes of intrahepatic cholestasis (e.g., drug-induced) and from mechanical obstruction of the intrahepatic bile ducts (e.g., pancreatitis, choledocholithiasis) before being accepted. Alcoholic hepatitis (steatonecrosis) is characterized by a constellation of lesions: steatosis, Mallory bodies (with or without a neutrophilic inflammatory response), megamitochondria, occlusive lesions of terminal hepatic venules, and a lattice-like pattern of pericellular fibrosis. All these lesions mainly affect zone 3 of the hepatic acinus. Other changes, observed at the ultrastructural level, are of importance in progression of the disease. They include widespread cytoplasmic shedding, and capillarization and defenestration of sinusoids. Progressive fibrosis complicating alcoholic hepatitis eventually leads to cirrhosis that is typically micronodular but can evolve to a mixed or macronodular pattern. Hepatocellular carcinoma occurs in 5 to 15% of patients with alcoholic liver disease. The clinical syndrome of alcoholic liver disease is the result of three factors--parenchymal insufficiency, portal hypertension and the clinical consequences of extrahepatic damage produced by alcohol. At the several phases of the life history of alcoholic liver disease, the individual factors play a different role. The clinical manifestations of alcoholic steatosis are mainly extrahepatic in origin. Those of alcoholic hepatitis reflect mainly parenchymal insufficiency and those of cirrhosis are mainly those of portal hypertension. Alcoholic liver injury appears to be generated by the effects of ethanol metabolism and the toxic effects of acetaldehyde, perhaps the immune responses to alcohol- or acetaldehyde-altered proteins, and questionably enhanced by viral hepatitis. Alcoholic hepatitis may be mimicked histologically, and to a varying degree clinically, by a number of conditions (obesity, diabetes, several drug-induced injuries, jejunoileal bypass, and related "shortcircuiting" of the bowel). Perhaps the most important facet of the hepatotoxicity of alcohol is its enhancement of the effects of a number of other hepatotoxic agents, among which acetaminophen is the prime example.
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PMID:Alcoholic liver disease: pathologic, pathogenetic and clinical aspects. 205 45

A retrospective analysis of 860 liver biopsy specimens processed by the Department of Pathology of Addis Ababa University was made to determine the frequencies of the various histopathological lesions seen among Ethiopians admitted with liver disease. One hundred fifty six (18.1%) of the specimens were inadequate for precise pathological diagnosis. Liver cirrhosis accounted for 25.4% (179) and primary hepatocellular carcinoma for 19.2% (135) of all diagnoses. Porphyria cutanea tarda was diagnosed in 12.4% (87) of the biopsy specimens. Hepatitis, metastases to the liver, and hepatic granulomata were present in 8.8% (62), 4.5% (32) and 2.8% (20) of the specimens respectively. In countries like Ethiopia where autopsies and diagnostic facilities are limited, and liver diseases prevalent, percutaneous needle biopsy is a useful procedure to define the histopathology of different types of liver disease.
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PMID:Histopathological features of liver disease in hospitalized Ethiopian patients. 292 Jul 10

A patient with alcoholic liver cirrhosis, diabetes mellitus and porphyria cutanea tarda (PCT) is described, who subsequently died of hepatocellular carcinoma. The literature relating PCT to the incidence of primary hepatoma is reviewed, and the mechanisms underlying this possible association are discussed.
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PMID:Is porphyria cutanea tarda a risk factor in the development of hepatocellular carcinoma? A case report and review of the literature. 298 17

In order to assess the incidence of hepatocellular carcinoma (HCC) in porphyria cutanea tarda (PCT), 83 patients (77 males, 6 females, mean age 57.4 years) were studied. Thirteen patients (15.7%) had HCC, all of whom were male and cirrhotics with a mean age of 58.5 years. HCC patients showed a statistically significant (P less than 0.0005) longer evolution time (23 years since onset of the cutaneous disease) than patients without HCC (9.4 years), while the age of onset was similar in both groups. Differences in alcohol intake and hepatitis B virus (HBV) markers were non-significant, although high prevalence (54%) of past HBV infection was found in both groups. In HCC development, attributable risks of 100% were found for cirrhosis (P less than 0.001), male sex (P = NS) and for age over 51 (P less than 0.025). Therefore, PCT harbours a high incidence of HCC; evolution time, cirrhosis and age over 51 appear to be the most important contributing factors.
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PMID:Porphyria cutanea tarda and hepatocellular carcinoma. Frequency of occurrence and related factors. 299 23

Our investigations in 134 patients showed corresponding to literature porphyria cutanea tarda (PCT) diagnosed by biochemical methods not to be a paraneoplastic dermatosis (but one possible exception acquainted). Relations between PCT and extrahepatic non-porphyrin producing tumours are improbable. Nevertheless but extremely seldom an irregular urinary porphyrin excretion associated with cutaneous changes of hepatic porphyria should lead to the presumption of a porphyrin producing hepatoma. PCT lasting for decades apparently presents a higher frequency of hepatocellular carcinoma in patients suffering from liver cirrhosis than in cirrhotics without PCT. It is supposed that this possible progredience of liver disease in PCT into hepatocellular carcinoma may be prevented by chloroquine phosphate therapy.
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PMID:[Does paraneoplastic porphyria cutanea tarda exist?]. 337 Dec 36

Primary liver cell carcinoma (PLC) is common in Africa. It represents the commonest malignancy in the Nigerian males. White reports of paraneoplastic syndrome associated with PLC abound from the Western World--a geographic area of low incidence of the tumour--there is a paucity of information from the African continent generally, and specifically from Nigeria. This prospective study analyses the relative incidences of recognized syndromes in fifty Nigerians with PLC. Alpha-foetoproteinemia (51%) and hypercholesterolaemia (33%) are common while hypoglycemia (9%) and hypercalcaemia (4%) are uncommon. No case of porphyria cutanea tarda was encountered. Our findings are compared with reported incidences in the literature. The pathogenetic mechanisms of these syndromes are briefly reviewed.
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PMID:Paraneoplastic syndromes in primary liver-cell carcinoma: experience in Ibadan, Nigeria. 617 79

We report the findings in 53 biopsies from 45 patients with porphyria cutanea tarda (PCT). Red autofluorescence and birefringent acicular cytoplasmic inclusions were constant findings in all untreated cases. Autofluorescence occurs in other hepatic porphyrias, but acicular inclusions appear to be specific for PCT; we have seen them in subclinical porphyria and before development of cutaneous symptoms. They are probably uroporphyrins and they trend to disappear during rinsing by water during most staining procedures. We recommend unstained paraffin sections for their demonstration. Liver damage in PCT has features distinct from other liver diseases, including alcoholic liver disease. These include constant but mild periportal siderosis, focal lipofuscin deposition, focal lobular hepatocyte necrosis associated with groups of pigment-laden macrophages, focal steatosis, marked hepatocyte hyperplasia and the presence of periductal lymphocyte aggregates. The latter have not been previously described in PCT and were present in 43% of our cases. There is a direct relationship between increasing age and progressive distortion of liver architecture, with fibrosis present at a mean age of 48 years, cirrhosis at 57 and hepatocellular carcinoma at 66. The characteristic liver histology and the natural history of PCT are against this being the result of any non-specific liver damage and favour instead a specific liver disease whose pathogenesis may be mainly the result of the metabolic defect of PCT.
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PMID:The pathology of the liver in porphyria cutanea tarda. 625 81


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