Hepatic ultrasonography in type I glycogen storage disease (von Gierke disease). Detection of hepatic adenoma and carcinoma.

Grossman, H. Barton; Ram, Panol C.; Coleman, Rosalind A.; Gates, Gary F.; Rosenberg, Eric R.; Bowie, J D; Wilkinson, R H

doi:10.1148/radiology.141.3.6272355

Cited by 36 publications

(11 citation statements)

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“…Mostly, the tumors in these patients were encapsulated and show a transformation to highly differentiated hepatocellular carcinomas from adenomatous hyperplasias 23 . Actually, both hepatocellular carcinoma and adenoma tissue could be found within the same tumor 24,25 . A previous study also reported the simultaneous appearance of adenoma and cancer 26 .…”

Section: Discussionmentioning

confidence: 72%

Glucose‐6‐phosphatase gene mutations in 20 adult Japanese patients with glycogen storage disease type 1a with reference to hepatic tumors

Nakamura

Ozawa

Kawasaki

et al. 2001

J of Gastro and Hepatol

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The G727T mutation seems to be common among Japanese patients with GSD1a, and the discovery of one heterozygote with a combination of G727T and G327A mutations (the latter mutation is common among Chinese) by the use of polymerase chain reaction-single strand conformation polymorphism analysis gave further insight into Japanese ancestry. This is the first study of liver tumors in a large group of adult GSD1a patients with the G727T mutation. As most of the patients in our series are free from other chronic liver diseases such as viral hepatitis, other genetic and/or acquired factors may have influence on the sequel to this metabolic disease.

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Section: Discussionmentioning

confidence: 72%

Glucose‐6‐phosphatase gene mutations in 20 adult Japanese patients with glycogen storage disease type 1a with reference to hepatic tumors

Nakamura

Ozawa

Kawasaki

et al. 2001

J of Gastro and Hepatol

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“…Adenomas are more common, usually appearing during or after the second decade of life. Although HCC is less common, it often occurs at an early age and in the absence of cirrhosis (105)(106)(107)(108)(109)(110). In several cases, progression of adenoma to HCC has been suggested by the clinical course (104)(105)(106)(107)(108).…”

Section: Type I Glycogen Storage Disease Fatty-acid Metabolism and Hmentioning

confidence: 99%

“…Although HCC is less common, it often occurs at an early age and in the absence of cirrhosis (105)(106)(107)(108)(109)(110). In several cases, progression of adenoma to HCC has been suggested by the clinical course (104)(105)(106)(107)(108). Second, continuous diurnal and nocturnal provision of exogenous carbohydrate in the form of starch or glucose (111) not only may improve the metabolic disturbance, including decreased mobilization of FFA from adipose tissue, but may also prevent, arrest the growth of or even cause regression of hepatic adenomas (1121, although exceptions have been noted (109).…”

Section: Type I Glycogen Storage Disease Fatty-acid Metabolism and Hmentioning

confidence: 99%

Fatty-acid metabolism and the pathogenesis of hepatocellular carcinoma: Review and hypothesis

Ockner

1993

Hepatology

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Despite increasing understanding of the genetic control of cell growth and the identification of several involved chemical and infectious factors, the pathogenesis of clinical and experimental hepatocellular carcinoma remains unknown. Available evidence is consistent with the possibility that selected changes in the hepatocellular metabolism of long-chain fatty acids may contribute significantly to this, process. Specifically, studies of the peroxisome proliferators, a diverse group of xenobiotics that includes the fibrate class of hypolipidemic drugs, suggest that increased fatty acid oxidation by way of extramitochondrial pathways (i.e., omega-oxidation in the smooth endoplasmic reticulum and beta-oxidation in the peroxisomes) results in a corresponding increase in the generation of hydrogen peroxide and, thus, oxidative stress. This in turn leads to alterations in gene expression and in DNA itself. We also review evidence supporting a potentially decisive influence of particular aspects of hepatocellular fatty acid metabolism in determining the activity of the extramitochondrial pathways. Moreover, certain intermediates of extramitochondrial fatty acid oxidation (e.g., the long-chain dicarboxylic fatty acids) impair mitochondrial function and are implicated as modulators of gene expression through their interaction with the peroxisome proliferator-activated receptor. Finally, the occurrence of hepatic tumors in type I glycogen storage disease (glucose-6-phosphatase deficiency) may exemplify this general mechanism, which may also contribute to nonneoplastic liver injury and to tumorigenesis in other tissues.

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“…While the tumors are relatively rare in the first decade of life they appear in all patients who pass through adolescence. Histologically both adenomas and carcinomas were diagnosed and in some cases the clinical course of the disease suggested transformation from adenomas into carcinomas (36,43,61,116). The increasing reports on the development of hepatic tumors in patients with inborn hepatic glycogenosis have been explained as a consequence of the supportive therapy which nowadays ensures that more and more infants with Type I gly- cogen storage disease are surviving into childhood and young adulthood (36).…”

Section: Relevance Of the Observations In Rodents To Hepatocarcinogenmentioning

confidence: 99%

“…Histologically both adenomas and carcinomas were diagnosed and in some cases the clinical course of the disease suggested transformation from adenomas into carcinomas (36,43,61,116). The increasing reports on the development of hepatic tumors in patients with inborn hepatic glycogenosis have been explained as a consequence of the supportive therapy which nowadays ensures that more and more infants with Type I gly- cogen storage disease are surviving into childhood and young adulthood (36). The observations in humans strongly support our hypothesis that the metabolic disturbance leading to the hepatocellular glycogenosis, which is probably fixed at the genetic level in both humans and experimental animals, might also be responsible for the neoplastic transformation of the hepatocytes (4,13).…”

Section: Relevance Of the Observations In Rodents To Hepatocarcinogenmentioning

confidence: 99%

Biological markers of preneoplastic foci and neoplastic nodules in rodent liver

et al. 1982

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Foci of altered hepatocytes are regularly observed early during hepatocarcinogenesis in rodents. The abnormal hepatocytes may show a number of different phenotypes as characterized by various cytomorphological and cytochemical markers. The first appearance and the further development of the abnormal cell populations depend on the dose of the carcinogen given and on the duration of the carcinogenic treatment. According to cytochemical, morphometric and autoradiographic findings in rats receiving low doses (2-10% of the LD 50/kg bw/ day) of hepatocarcinogens for limited periods (iistop" experiments), glycogenotic (clear or acidophilic) hepatocytes indicate the first step of the neoplastic cell transformation which can be detected by these methods at present. The glycogenotic cells undergo a characteristic metamorphosis and give rise to basophilic tumor cells poor in glycogen, but rich in ribosomes. Under extreme experimental conditions, such as a single or repeated application of higher doses of one or several chemical carcinogens a puzzling picture emerges which is "reversible" to a large extent after withdrawal of the respective compounds. This observation points to a phenotypic instability of the cellular changes induced in certain experimental systems. Foci of altered hepatocytes persisting after withdrawal of the carcinogenic compounds are considered preneoplastic lesions. They may transform into neoplastic nodules which are also persistent and share a number of cytomorphological and cytochemical markers with the focal lesions. The persistent nodules progress to hepatocarcinomas after lag periods of weeks or months. However, the foci may also progress to hepatocarcinomas without passing a nodular intermediate stage. The development of both neoplastic nodules and carcinomas from the preneoplastic glycogen storage foci can proceed independent of further administration of carcinogen. The sequence of cellular changes during hepatocarcinogenesis derived from the experimental results in rodents is strongly supported by observations in humans, especially by the increasing reports on the appearance of hepatic tumors in patients who suffer from inborn hepatic glycogenosis.

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Hepatic ultrasonography in type I glycogen storage disease (von Gierke disease). Detection of hepatic adenoma and carcinoma.

Cited by 36 publications

References 0 publications

Glucose‐6‐phosphatase gene mutations in 20 adult Japanese patients with glycogen storage disease type 1a with reference to hepatic tumors

Glucose‐6‐phosphatase gene mutations in 20 adult Japanese patients with glycogen storage disease type 1a with reference to hepatic tumors

Fatty-acid metabolism and the pathogenesis of hepatocellular carcinoma: Review and hypothesis

Biological markers of preneoplastic foci and neoplastic nodules in rodent liver

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