Hepatic hemangioma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

Overview

Development of hepatic hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis. Hepatic hemangioma may be associated with Kasabach-Merritt syndrome, hereditary hemorrhagic telangiectasia, hepatic arterio-portal shunts, extra hepatic hemangiomata, hemolytic anemia, focal nodular hyperplasia. On gross pathology, variable in size, well circumscribed, and classically subcapsular are findings of hepatic hemangioma. On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.

Pathogenesis

The pathogenesis of hepatic hemangiomas has not been elucidated, but there are two competing theories.

First theory

• The first theory supports the notion that hepatic hemangioma may be the result of overexpression of angiogenic factors and downregulation of inhibitors of angiogenesis.

• Overexpression of angiographic factors, such as:

• Vascular endothelial growth factor
• Basic fibroblast growth factor
• Metalloproteinases

• Downregulation of inhibitors of angiogenesis, such as:

• Tissue inhibitor of metalloproteinase-I

Second theory

• The second theory suggests that the presence of liver hemangiomas involves a genetic background of mutations.

• Metalloproteinases accumulate in the endoplasmic reticulum of the tumor cells. Accumulation may result in the following:

• Self-digestion
• Vacuole formation

• Cavernous hemangioma cell to downregulate Derlin-1.

• Derlin-1 is a protein encoded by DERL1. When overexpressed, derlin-1 protein induces the dilated endoplasmic reticulum to return to its normal size.

Associated Conditions

Hepatic hemangioma may be associated with:

• Kasabach-Merritt syndrome
• Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
• Hepatic arterio-portal shunts
• Extra hepatic hemangiomata
• Hemolytic anemia
• Consumptive coagulopathy
• Focal nodular hyperplasia

Gross Pathology

On gross pathology, a well circumscribed, subcapsular tumor may be suggestive of hepatic hemangioma.

Microscopic Pathology

On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.

Gallery

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  Intermediate magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.

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  High magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.