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Infantile Hepatic Hemangioendothelioma (IHE) is a vascular tumor of endothelial origin, often considered part of the spectrum of hepatic hemangiomas.

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• Most common benign hepatic tumor of infancy, usually presenting in the first 6 months of life.
• Can be solitary or multifocal.

Etiopathology

• Thought to arise from developmental anomaly of hepatic vasculature.
• May be associated with:
  • Cutaneous hemangiomas (infantile hemangiomatosis).
  • Kasabach–Merritt syndrome (consumptive coagulopathy, thrombocytopenia).
  • High-output cardiac failure (due to AV shunting).
• Rare malignant transformation into angiosarcoma has been described.

Clinical Features

• Usually presents in first year of life.
• Abdominal mass, hepatomegaly.
• Cutaneous hemangiomas.

![Multiple cutaneous IHs associated with MHHs in a 1-month-old girl (A) Multiple IHs on the face and trunk. This patient also had additional lesions on the extremities. (B) T2-weighted axial MRI of the liver showed lesions were hyperintense with intervening areas of normal hepatic parenchyma.

Ji, Y., Chen, S., Xiang, B. et al. Clinical features and management of multifocal hepatic hemangiomas in children: a retrospective study. Sci Rep 6, 31744 (2016). https://doi.org/10.1038/srep31744](attachment:cd3fb8dc-6f7f-41aa-afff-c83f51c4c1ab:image.png)

Multiple cutaneous IHs associated with MHHs in a 1-month-old girl (A) Multiple IHs on the face and trunk. This patient also had additional lesions on the extremities. (B) T2-weighted axial MRI of the liver showed lesions were hyperintense with intervening areas of normal hepatic parenchyma.

Ji, Y., Chen, S., Xiang, B. et al. Clinical features and management of multifocal hepatic hemangiomas in children: a retrospective study. Sci Rep 6, 31744 (2016). https://doi.org/10.1038/srep31744

Complications:

• High-output cardiac failure (due to intra-tumoral AV shunts).
• Consumptive coagulopathy (Kasabach–Merritt phenomenon (KMP) (hemangioma thrombocytopenia syndrome) ).
• Hypothyroidism (due to type 3 deiodinase expression in tumor).

Imaging Findings