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Glycogen storage disease I and hepatocellular tumours

  • Treatment and complications in GSD type Ib
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Abstract

From 50 published cases of hepatocellular adenoma (HCA) in glycogen storage disease, type I (GSD I) some characteristic features may be deduced: 1. The male: female ratio was 2∶1. This sharply contrasts to HCA of other origin which shows a strong female preponderance. 2. The histology of adenomas largely corresponded to other adenomas, except for the appearance of Mallory bodies, accompanied by neutrophilic inflammation and a peculiar lamellar fibrosis. This observation is of particular interest because Mallory bodies have so far not been described in adenomas but are a well established feature in hepatocellular carcinoma of any aetiology. 3. Adenomas had a tendency to regress after continuous nocturnal intragastric feeding, although not all cases responded favourably. 4. Ten cases of hepatocellular carcinoma (HCC) are recorded in GSD I in literature, whereby in half of them transition from HCA into HCC seems likely. 5. The similarity in clinical presentation and evolution with oestrogen-induced tumours is striking. Pathogenesis of adenoma formation in GSD I is not understood. Experimental evidence and the clinical observation of regression after correction of the metabolic imbalance suggest three possible candidate mechanisms: (1) a glucagon/insulin imbalance; (2) cellular glycogen overload; and (3) proto-oncogene activation. Evidence in favour of these three mechanisms from experimental studies and observations in humans are briefly reviewed.

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Abbreviations

GSDI:

glycogen storage disease, type I

HCA:

hepatocellular adenoma

HCC:

hepatocellular carcinoma

FNH:

focal nodular hyperplasia

IGF:

(nocturnal) intragastric feeding

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  1. Department of Pathology, University of Basel, Schönbeinstrasse 40, CH-4003, Basel, Switzerland

    L. Bianchi

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Bianchi, L. Glycogen storage disease I and hepatocellular tumours. Eur J Pediatr 152 (Suppl 1), 63–70 (1993). https://doi.org/10.1007/BF02072092

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