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Updated: Jan 23, 2026

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Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma

Ahmed Emad1, Sayed Fadel1,2, Madeeha El Wakeel3

  • 1Departments of Pediatric Hematology and Oncology.

Journal of Pediatric Hematology/Oncology
|June 25, 2019
PubMed

Insights

Infantile hepatic hemangioendothelioma (IHHE) is common in children. Many IHHE patients, especially those with focal lesions, do not require drug treatment and have good outcomes.

Area of Science:

  • Pediatric Oncology
  • Hepatology
  • Vascular Malformations

Background:

  • Infantile hepatic hemangioendothelioma (IHHE) is the most frequent hepatic vascular tumor in pediatric populations.
  • Early diagnosis and management are crucial for improving patient outcomes.

Purpose of the Study:

  • To evaluate the treatment outcomes of infantile hepatic hemangioendothelioma (IHHE) in a large single-center cohort.
  • To identify factors influencing treatment response and prognosis in IHHE patients.

Main Methods:

  • Retrospective analysis of 28 IHHE patients treated between April 2008 and April 2017.
  • Categorization of lesions as multifocal, focal, or diffuse.
  • Assessment of treatment strategies including observation, surgery, and medical therapy (propranolol/prednisolone).

Main Results:

  • A total of 28 patients (18 females, 10 males) diagnosed with IHHE, with a median age of 3 months.
  • Lesion types included multifocal (n=12), focal (n=10), and diffuse (n=6).
  • Eleven patients received no treatment, 1 underwent surgery, and 16 received drug therapy; 9 responded to first-line treatment, and 7 required salvage therapy. Twenty-five patients survived, and 3 died.

Conclusions:

  • Patients with IHHE generally experience favorable outcomes.
  • A significant proportion of IHHE cases, particularly focal lesions, do not necessitate drug intervention.
  • Multifocal/diffuse disease is associated with a higher incidence of hypothyroidism (low T3 and T4), and rapidly progressing lesions may indicate a poorer prognosis.
Abstract

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