Abstract
Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy, but is rarely reported because of the low incidence estimated to be about 1/20,000. We report an autopsy case of IHHE in a 20-day-old female who came with an initial manifestation of abdominal distension and congestive heart failure (CHF). Symptoms of cardiac decompensation gradually worsened in spite of treatment and child died within 2 days. On autopsy, grossly enlarged liver showed microscopy of large ecstatic vascular channels lined by single layer of plump endothelial cells and diagnosis was given as Type I IHHE. CHF is common complication due to arteriovenous shunts within the tumor and contributes to high morbidity and mortality in up to 70% of untreated infants with median age of presentation of 1-month. Therefore, if symptoms develop, aggressive treatments are warranted.
Published Version
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