Abstract
Of the many complications associated with glycogen storage disease type I, hepatic tumours cause great concern because of their malignant potential and the current difficulties in monitoring them. Hepatic adenomas occur in 22%–75% of affected adults, according to the population studied, and from those reported in the literature are thought to have an approximately 10% risk of undergoing malignant transformation. Their aetiology is unclear, but they occur generally in postpubertal patients, and can be either single or multiple. This article discusses theories of their aetiology, methods of detection and monitoring, and treatment options.Conclusion: the incidence of liver tumours in younger adults seems less than in older ones, suggesting that better dietary treatment, and thus improved metabolic control, may be protective. Surgery (partial hepatectomy or orthotopic liver transplantation) is the definitive therapy for these tumours, but the timing of this intervention is difficult to determine and it is not without its own hazards.
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Abbreviations
- GSD-I :
-
glycogen storage disease type I
References
Bianchi L (1993) Glycogen storage disease I and hepatocellular tumours. Eur J Pediatr 152[Suppl 1]: S63-S70
Conti JA, Kemeny N (1992) Type Ia glycogenosis associated with hepatocellular carcinoma. Cancer 69: 1320–1322
De Parscau L, Guibaud P, Labrune P, Odievre M (1988) Évolution à long terme des glycogenoses hépatiques. Arch Franç Pediatr 45: 641–645
Labrune P, Trioche P, Duvaltier I, Chevalier P, Odievre M (1997) Hepatocellular adenomas in glycogen storage disease type I and III: a series of 43 patients and review of the literature. J Pediatr Gastroenterol Nutr 24: 276–279
Lee P, Mather S, Owen C, Leonard J, Dicks-Mireaux C (1994). A variety of hepatic ultrasound findings exist in the hepatic glycogen storage diseases. Brit J Radiol 67: 1062–1066
Lee PJ, Leonard JV, Dicks-Mireaux C (1995) Focal fatty liver change in glycogenosis type Ia. Eur J Pediatr 154: 332
Limmer J, Fleig WE, Leupold D, Bittner R, Ditschneit H, Berger H-G (1988) Hepatocellular carcinoma in type I glycogen storage disease. Hepatology 8: 531–537
Mason HH, Andersen DH (1955) Glycogen disease of the liver (von Gierke's disease) with hepatomata: case report with metabolic studies. Pediatrics 16: 785–799.
Matern D, Starzl TE, Arnaout W, Barnard J, Bynon J, Dhawan A, Emond J, Haagsma EB, Hug G, Lachaux A, Smit GPA, Chen Y-T (1999) Liver transplantation of glycogen storage disease types I, III, and IV. Eur J Pediatr 158[Suppl 2]: S43-S48
Ockner RK, Kaikaus RM, Bass NM (1993) Fatty-acid metabolism and the pathogenesis of hepatocellular carcinoma: review and hypothesis. Hepatology 18: 669–676
Parker P, Burr I, Slonim A, Ghishan FK, Greene H (1981) Regression of hepatic adenomas in type Ia glycogen storage disease with dietary therapy. Gastroenterology 81: 534–536
Smit GPA, Fernandes J, Leonard JV, Matthews EE, Moses SW, Odievre M, Ullrich K (1990) The long-term outcome of patients with glycogen storage diseases. J Inherit Metab Dis 13: 411–418
Talente GM, Coleman RA, Alter C, Baker L, Brown BI, Cannon RA, Chen Y-T, Crigler JF, Ferreira P, Haworth JC, Herman GE, Issenman RM, Keating JP, Linde R, Roe TF, Senior B, Wolfsdorf JI (1994) Glycogen storage disease in adults. Ann Intern Med 120: 218–226
Wolfsdorf JI, Gutierrez ME, Crigler JF (1994) Determinants of focal hepatic lesions in type I glycogenosis (GSD-I). Pediatr Res 35: 209A
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Published online: 12 July 2002
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Lee, P.J. Glycogen storage disease type I: pathophysiology of liver adenomas. Eur J Pediatr 161, S46–S49 (2002). https://doi.org/10.1007/BF02679993
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DOI: https://doi.org/10.1007/BF02679993