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

The predisposition of preexisting cirrhotic metabolic liver disease to the development of hepatocellular carcinoma is well established. However, the association between glycogenoses (a form of noncirrhotic metabolic liver disease) and the formation of benign and malignant liver tumors is less well known. The sixth case of simultaneous occurrence of glycogenosis type Ia and hepatocellular carcinoma, which occurred in a 34-year-old man referred for advice on therapy for his malignant neoplasm, is reported. Cases previously described in the literature are reviewed and management of this entity in light of the current knowledge in the diagnosis and treatment of hepatocellular carcinoma is discussed.
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PMID:Type Ia glycogenosis associated with hepatocellular carcinoma. 131 20

The benign tumors hepatic adenoma and focal nodular hyperplasia are compared in their etiology, differential diagnosis, risk of transformation, and management. Hepatic adenomas range in size from 1-30 cm, averaged 8-10 cm in diameter, contain vacuoles and glycogen, but no Kupfer cells or bile ducts. Adenoma is usually symptomatic, causing pressure or hemorrhage. The risk of developing adenoma is increased with duration of oral contraceptive use, and chance of a larger tumor, a hemorrhage and mortality during pregnancy or surgery is also increased in pill users. Adenoma also occurs in people with Type Ia glycogen storage disease, and is associated with insulin-dependent diabetes. Often stopping oral contraceptives will cause an adenoma to regress. If not, It is best managed by elective resection, with 1% mortality, rather than 5-10% mortality due to spontaneous rupture. Adenomas can progress to adenomatosis, which are inoperable, or malignant transformation. Focal nodular hyperplasia is marked by a stellate scar, sometimes accompanied by hemangioma, but is asymptomatic. It is not increased in oral contraceptive users, but occurs in older women. It can transform to fibrolamellar hepatocellular carcinoma. The 2 benign lesions can be distinguished by radionuclide scanning and angiography. Only fine needle aspiration is advised for biopsy, because of the risk of hemorrhage with adenoma. Focal nodular hyperplasia takes up radionuclide, stains intensely on angiography, and is safe to biopsy percutaneously.
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PMID:Hepatic adenoma and focal nodular hyperplasia. 165 55

We report 2 cases of type I glycogen storage disease (Von Gierke's disease) discovered in 2 brothers at the age of 7 and 5 years, respectively. Both developed hepatic adenoma at the age of 19 and 17. Hepatocellular carcinoma occurred in the older brother the discovery of adenoma 4 years after. The frequency of these tumors in patients with type I glycogen storage disease raises problems concerning the treatment and modality of regular surveillance of the liver in these patients. The policy for the detection and treatment of these tumors, and particularly the indications for liver transplantation are discussed.
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PMID:[Hepatic adenoma and hepatocellular carcinoma in 3 brothers with type I glycogenosis]. 215 41

Clinical and histologic details of the two siblings with type 1a glycogen storage disease (GSD-1a) who developed hepatoblastoma are presented. The light microscopic studies on hepatic tumor in both siblings revealed fetal type of hepatoblastoma. Ultrastructural findings in Patient 2 showed markedly altered mitochondria, which were frequently surrounded by the rough endoplasmic reticulum. This is the first known occurrence with this association, and the third report on the familial occurrence of this neoplasm. Glycogen storage disease may increase the risk of hepatocellular carcinoma and hepatoblastoma.
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PMID:Type 1a glycogen storage disease with hepatoblastoma in siblings. 303 May 27

Twenty-four patients with liver cell adenomas were referred to Paul Brousse Hospital between 1976 and 1987. This represents the largest reported surgical experience of this pathology from a single centre. Six patients had multiple adenomas, which were associated with glycogen storage disease in four. Two patients had polyadenomatosis, one of whom underwent successful liver transplantation after malignant transformation to hepatocellular carcinoma. Eighteen patients (median age of 33 years, range of 17-45 years) had either a solitary adenoma or two adenomas. Eighty-three per cent were women 87% of whom had received oral contraceptives or other hormone therapy before diagnosis (median duration of 11 years, range of 3-15 years). Fifty per cent of these patients presented with acute hemorrhage into an adenoma. Seventeen patients underwent surgical resection of their adenomas, with the remaining patient currently being treated by arterial embolizations to reduce the tumor size before surgery. There was no operative mortality or serious morbidity, and all patients remain well upon follow-up. Surgical excision of liver adenomas, where this can be done without causing mortality, is recommended. Resection relieves symptoms and removes both the risks of hemorrhaging into the tumour and of malignant transformation to hepatocellular carcinoma.
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PMID:Liver cell adenomas. A 12-year surgical experience from a specialist hepato-biliary unit. 319 Feb 82

Nocturnal intragastric feeding of patients with certain hepatic forms of glycogen storage disease has been advocated as an effective treatment, resulting in improved blood chemical values and linear growth. We are reporting the long-term follow-up of five patients with glycogen storage diseases; three with type Ia, one with type Ib, and one with type III disease. All had improvement in one or more of the following: linear growth, serum glutamic oxaloacetate transaminase, total lipids, cholesterol, phospholipids, or triglycerides. None had significant improvement in venous CO2, serum lactate or urate. One of the patients in this study died after 1.1 years of treatment, and another patient developed hepatocellular carcinoma after 4.4 years of treatment. Nocturnal intragastric feeding, in conjunction with appropriate daytime feeding, is helpful in the management of patients with glycogen storage disease but response to treatment is variable, and it remains to be determined whether the ultimate prognosis of the diseases can be improved.
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PMID:Glycogen storage disease: long-term follow-up of nocturnal intragastric feeding. 680 87

As a part of a routine yellow fever surveillance program going on in the south of Bahia State, Brazil, liver fragments were obtained through postmortem viscerotomy from 702 individuals who died after presenting acute febrile illness from 1981 up till 1991. Instead of being only screened for the presence of yellow fever, the liver tissue was thoroughly evaluated by histopathology. More than a third of the cases exhibited marked and diffuse steatosis occurring in malnourished infants and young children. Hepatic fibrosis, granulomatous disease compatible with disseminated tuberculosis, advanced schistosomiasis, chronic alcoholic injury, chronic hepatitis and cirrhosis were also frequently observed. A miscellaneous group of hepatic pathological processes were also recognized, which included such diverse entities as Hodgkin's disease, glycogenosis, sickle-cell disease, hepatocarcinoma, etc. Only 124 (17.7%) cases showed normal hepatic histology. The wide possibility of histological diagnoses strongly indicates that the material obtained by viscerotomy can be further explored by an interested pathologist, to help in the understanding of nosology and epidemiology, concerning remote geographic areas where viscerotomy is being routinely performed.
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PMID:[Hepatic viscerotomy (its contribution to the study of regional nosology)]. 752 Oct 56

The clinicopathological findings of eight children with hepatic adenoma in the absence of cirrhosis are presented. The lesions ranged in diameter from 0.1 to 14.5 cm. Associated disorders were Fanconi's anemia, type I glycogen storage disease. Hurler's disease, and severe combined immunodeficiency with ADA deficiency. The remaining three children had adenoma without known associated disorders. In the children with glycogenosis and Hurler's disease the adenomas were multiple. Significant dysplasia occurred in the two children with Fanconi's anemia; however, the lesions behaved in a benign fashion--one with regression of the tumor after cessation of androgen therapy and the other with nonrecurrence after complete resection. Proliferating cell nuclear antigen (PCNA) labeling index (LI) of the adenoma arising in patients with Fanconi's anemia was significantly greater than the PCNA-LI of adenoma in the other children (mean 4.1% versus 0.9% of nuclei), approaching the lower end of the spectrum for reported hepatocellular carcinoma cases. We emphasize that the worrisome pathology that may occur in hepatic adenoma in children, particularly with Fanconi's anemia, does not necessarily predict malignant behavior. The association of hepatic adenoma with Hurler's disease or severe combined immunodeficiency has not been reported previously.
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PMID:Hepatic adenoma in the pediatric age group. Clinicopathological observations and assessment of cell proliferative activity. 757 76

Hepatocarcinogenesis in hepatitis B virus transgenic mice was studied by means of a correlative cytomorphological and cytochemical approach at different time points in animals from 1 to 34 mo old. HBsAg-positive ground-glass hepatocytes emerged throughout the liver parenchyma in nearly all transgenic mice during the first 4 mo after birth. The panlobular expression of HBsAg persisted until foci of altered hepatocytes appeared (6 to 9 mo of age). Three different types of foci of altered hepatocytes-namely, glycogen-storage foci, mixed cell foci and glycogen-poor foci-developed. Hepatocellular adenomas and carcinomas appeared after 11 mo. Orcein staining revealed frequent transitions between ground-glass hepatocytes extensively expressing HBsAg and glycogen-storage (predominantly clear-cell) foci containing HBsAg-positive cytoplasmic components. Similar transitions between ground-glass hepatocytes and glycogenotic (clear) cells were often found in diffuse parenchymal glycogenosis at 11 or 12 mo. Remnants of HBsAg-positive material were also detected in mixed cell foci, glycogen-poor diffusely basophilic cell foci, hepatic adenoma and hepatocellular carcinoma. These findings suggest that ground-glass hepatocytes are the direct precursor of foci of altered hepatocytes and their neoplastic descendants. The extensive expression of HBsAg is gradually down-regulated during neoplastic transformation, just as the morphological the biochemical phenotypes of foci of altered hepatocytes, hepatic adenoma and hepatocellular carcinoma in transgenic mice resemble those described in chemical hepatocarcinogenesis. The predominant sequence of cellular changes leading from glycogen-storage (predominantly clear cell) foci to mixed cell foci, hepatic adenoma and hepatocellular carcinoma is characterized by a gradual decrease in the activities of glycogen synthase, phosphorylase, glucose-6-phosphatase and adenylate cyclase, whereas glucose-6-phosphate dehydrogenase and pyruvate kinase activities increase. These alterations indicate a shift from the glycogenotic state toward an increase in the pentose phosphate pathway and glycolysis.
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PMID:Hepatic preneoplasia in hepatitis B virus transgenic mice. 792 48

Preneoplastic and neoplastic hepatic lesions were induced in male Sprague-Dawley rats by oral exposure to N-nitrosomorpholine (12 mg/kg body wt/day) for 7 weeks (stop model). Twelve, 23 and 34 weeks after withdrawal of the carcinogen, cell proliferation and cell death (apoptosis) were studied in defined phenotypes of preneoplastic foci of altered hepatocytes (FAH), hepatocellular adenomas (HCA) and carcinomas (HCC) by autoradiographic determination of the labelling index (LI) resulting from continuous administration of [3H]thymidine for 48 h and by simultaneous counting of apoptotic bodies respectively. Compared with the liver parenchyma of untreated controls and the extrafocal parenchyma of treated animals, the mean LI was elevated in all types of FAH, HCA and HCC, but the extent of this increase differed markedly between the diverse phenotypes. The increase in the LI was significant for clear/acidophilic, intermediate and mixed/basophilic cell foci, but remained insignificant for the relatively rare tigroid and amphophilic cell foci. The previously established progression-linked phenotypic instability in the predominant cell lineage leading to HCC was associated with a gradual increase in the mean LI showing four significantly different proliferative stages: (i) clear/acidophilic and intermediate cell foci excessively storing glycogen, (ii) mixed/basophilic cell populations in FAH and glycogen-storing HCA, (iii) glycogen-poor HCA and glycogen-storing HCC and (iv) glycogen-poor HCC. The inverse correlation between glycogen accumulation and cell proliferation during progression from glycogenotic FAH to glycogen-poor HCC indicates that the fundamental metabolic shift associated with the gradual disappearance of the glycogenosis is essential for the evolution of the malignant phenotype. The mean ratio of necrotic cells (RN) was somewhat higher in all types of FAH compared to the normal and extrafocal liver parenchyma, but this was not statistically significant. Only when HCA and HCC appeared was there a significant increase in the mean RN, proceeding with the progression of neoplastic development. Our results do not support the concept that cell death (apoptosis) plays a major role in counterbalancing cell replication in FAH, but rather suggest that cell death occurs more frequently in the course of hepatocarcinogenesis the more neoplastic development advances.
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PMID:Cell proliferation and cell death (apoptosis) in hepatic preneoplasia and neoplasia are closely related to phenotypic cellular diversity and instability. 795 93


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