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[Diffuse infantile hepatic hemangioendothelioma: a case report].
Ivica Sjekavica1, Marko Petrovecki2, Vice Sunjara2
1Klinicki zavod za dijagnosticku i intervencijsku radiologiju, Medicinski fakultet Sveucilista u Zagrebu, KBC Zagreb. ivica.sjekavica@zg.htnet.hr
Lijecnicki Vjesnik
|December 25, 2013
Summary
Infantile hepatic hemangioendothelioma (IHHE) is a common pediatric liver tumor. In rare, aggressive cases, early liver transplantation may be life-saving when tumors resist standard therapy.
Area of Science:
- Pediatric Oncology
- Hepatology
- Vascular Anomalies
Background:
- Infantile hepatic hemangioendothelioma (IHHE) is the most frequent benign liver tumor in infants, typically responding well to treatment.
- While generally having low mortality, rare diffuse IHHE cases can become therapy-resistant, posing significant risks.
Observation:
- A 3-month-old infant presented with respiratory distress and an abdominal mass.
- Imaging (CT, MRI, DSA) and biopsy confirmed diffuse hemangioendothelioma resistant to medical therapy.
Findings:
- The infant's tumor grew, leading to cardiorespiratory failure and death at 13 months.
- This case highlights the potential for IHHE to be fatal despite initial presentation with common symptoms.
Implications:
- For diffuse, therapy-resistant IHHE, early consideration of liver transplantation is crucial.
- This underscores the need for vigilant monitoring and timely intervention in aggressive pediatric liver tumors.
