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Fatty liver disease
Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration
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Glycogen storage disease
No history of diabetes mellitus
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Hepatic glycogenosis
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Diabetes mellitus-associated glycogen storage hepatomegaly
Liver changes accompanied by growth retardation, delayed puberty, hypercholesterolemia, and cushingoid features
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Excessive glycogen storage in hepatocytes secondary to poorly controlled insulin-dependent diabetes mellitus
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Chronic hyperglycemia due to poorly controlled insulin-dependent diabetes mellitus
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Longstanding high blood sugar levels lead to glycogen accumulation in hepatocytes
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Hepatomegaly and transaminase elevations attributed to this excess glycogen accumulation
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Age
Occurs in both children and adults
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History of ketoacidosis
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Alkaline phosphatase may also be elevated
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Elevated hemoglobin A1c (HbA1c) indicates history of poor glycemic control
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Options, risks, complications
Mainstay treatment is improved management of diabetes mellitus
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Optimization of glycemic control with insulin and diet
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Surgical approaches
Resolution after pancreas transplantation has been reported
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Excellent outcome with medical management
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Liver histology improves and transaminases normalize with optimization of glycemic control
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Hyperdense liver on CT scan without administration of contrast material
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Diffuse pale-staining hepatocyte cytoplasm
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PAS stain confirms excessive glycogen accumulation in hepatocytes
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Rare acidophil bodies may be seen
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Glycogenated hepatocyte nuclei
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Notable absence of inflammation or other features of hepatic injury
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Marked glycogen accumulation in hepatocyte cytoplasm and nuclei
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Pale, enlarged hepatocytes may be mistaken for normal or interpreted as fixation artifact
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Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration
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Most cases of glycogenic hepatopathy show little or no steatosis
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Typically presents at younger age and without history of diabetes mellitus
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Marked transaminase elevation attributed to excessive glycogen accumulation in hepatocytes
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Consider in patients with poorly controlled diabetes mellitus and unexplained hepatomegaly &/or transaminase elevations
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Pale, slightly swollen hepatocytes with prominent cell membranes
May be mistaken for normal hepatocytes, glycogen storage disease, or fixation artifact
1.Sweetser, S, et al. The bright liver of glycogenic hepatopathy. Hepatology . 2010; 51(2):711–712.
2.Fridell, JA, et al. Complete reversal of glycogen hepatopathy with pancreas transplantation: two cases. Transplantation . 2007; 83(1):84–86.
3.Torbenson, M, et al. Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus. Am J Surg Pathol . 2006; 30(4):508–513.
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Diagnostic Pathology Hepatobiliary and Pancreas
. Glycogenated hepatocyte nuclei 
are common.
. They contain their normal structures, and there are no portal inflammatory cell infiltrates. The background hepatocytes 
are diffusely swollen and filled with glycogen.
