Hepatic Adenomata With Type 1 Glycogen Storage Disease

Howell, R. Rodney; Stevenson, Roger E.; Ben‐Menachem, Yoram; Phyliky, Robert L.; Berry, D. H.

doi:10.1001/jama.1976.03270140033019

Cited by 128 publications

(29 citation statements)

References 5 publications

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“…The relationship between good metabolic control and the appearance of HCA also remains to be de¢ned, although some reports suggest a lower incidence (Matern et al 1999) and even regression of the tumors (Parker et al 1981) in adequately treated patients. Recent follow-up studies suggest a prevalence of about 50%, similar to that described in the past (Coire et al 1987;Howell et al 1976;Weinstein and Wolfsdorf 2002). In the general population, HCA is associated with the use of oral contraceptives or exogenous androgens and tumors tend to be large, single and encapsulated (Dienstag and Isselbacher, 2005).…”

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confidence: 61%

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Hepatocellular carcinoma in glycogen storage disease type Ia: A case series

Franco

Krishnamurthy

Bali

et al. 2004

J of Inher Metab Disea

192

148

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We present a series of 8 patients (6 males, 2 females) with hepatocellular carcinoma (HCC) and glycogen storage disease type Ia (GSD Ia). In this group, the age at which treatment was initiated ranged from birth to 39 years (mean 9.9 years). All patients but one were noncompliant with treatment. Hepatic masses were first detected at an age range of 13-45 years (mean 28.1 years). Age at diagnosis of HCC ranged from 19 to 49 years (mean 36.9 years). Duration between the diagnosis of liver adenomas and the diagnosis of HCC ranged from 0 to 28 years (mean 8.8 years, SD = 11.5). Two patients had positive hepatitis serologies (one hepatitis B, one hepatitis C). Alpha-fetoprotein (AFP) was normal in 6 of the 8 patients. Carcinoembryonic antigen (CEA) was normal in the 5 patients in which it was measured. Current guidelines recommend abdominal ultrasonography with AFP and CEA levels every 3 months once patients develop hepatic lesions. Abdominal CT or MRI is advised when the lesions are large or poorly defined or are growing larger. We question the reliability of AFP and CEA as markers for HCC in GSD Ia. Aggressive interventional management of masses with rapid growth or poorly defined margins may be necessary to prevent the development of HCC in this patient population.

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confidence: 61%

“…Hepatocellular adenomas (HCAs) are present in most patients with GSD I by the second or third decade of life (Chen 2001;Howell et al 1976). The reported prevalence ranges from 22% to 75% and they mostly appear during or after puberty (Chen 2001;Lee 2002).…”

mentioning

confidence: 99%

Hepatocellular carcinoma in glycogen storage disease type Ia: A case series

Franco

Krishnamurthy

Bali

et al. 2004

J of Inher Metab Disea

192

148

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“…There are case reports of an adenoma progressing to malignancy (Howell et al, 1976) and of hepatocellular carcinoma coexisting with focal nodular hyperplasia in one female patient (Davis et al, 1975). Various theories of the mechanism of proliferation have been suggested.…”

Section: Commentmentioning

confidence: 99%

Survey of primary liver tumors and oral contraceptive use

Vána

Murphy

Aronoff

et al. 1979

Journal of Toxicology and Environmental Health

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The American College of Surgeons' survey data on 378 female and 165 male cases of primary liver tumors reported by 477 hospitals in the United States during 1970--1975 are presented. In males, 91.5% of the tumors were malignant, confirming the rarity of benign liver tumors in males. Among females, 43.9% were malignant and 56.1% were benign. Of the 212 benign tumors, 96 were hepatic cell adenomas and 58 were focal nodular hyperplasias. A positive history of oral contraceptive use was found in nearly half of all tumors, 65% of benign tumors, 74% of hepatic cell adenomas, and 74% of focal nodular hyperplasias. High frequencies of benign tumors were observed in the age group 20--30 yr. More than 80% of the tumors in this age group were found in oral contraceptive users. Symptomatology was more severe among users. No case of intraperitoneal bleeding was observed in nonusers. The findings confirm the suggested association between use of oral contraceptives and hepatic cell adenomas and focal nodular hyperplasias.

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“…Diagnostic difficulties concern particularly small adenomas. In addition to the typical forms of adenoma, HA may occur in the context of glycogenosis or following exposure to androgens [10,11].…”

Section: Introductionmentioning

confidence: 99%

Genetic alterations in hepatocellular adenomas: recent findings and new challenges

Zucman–Rossi

2004

Journal of Hepatology

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Hepatic Adenomata With Type 1 Glycogen Storage Disease

Cited by 128 publications

References 5 publications

Hepatocellular carcinoma in glycogen storage disease type Ia: A case series

Hepatocellular carcinoma in glycogen storage disease type Ia: A case series

Survey of primary liver tumors and oral contraceptive use

Genetic alterations in hepatocellular adenomas: recent findings and new challenges

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