Related Experiment Video
Updated: Jul 11, 2026
Laparoscopic Anatomical Right Hemihepatectomy via the In Situ Anterior Approach
Published on: August 8, 2025
Infantile hepatic hemangioendothelioma: clinical presentation and treatment
1Department of Pediatric Oncology, Uludağ University, School of Medicine, Bursa. bsevinir@yahoo.com
Insights
Infantile hepatic hemangioendothelioma (IHEH) is a rare childhood tumor. This study highlights treatment strategies and outcomes, showing an 87% survival rate with a multidisciplinary approach.
Area of Science:
- Pediatric Oncology
- Hepatology
- Vascular Tumors
Background:
- Infantile hepatic hemangioendothelioma (IHEH) is a rare benign vascular tumor of the liver in infants.
- Early diagnosis and management are crucial due to potential complications like high-output heart failure and Kasabach-Merritt syndrome.
- This study reviews a decade of experience with IHEH at a tertiary care center.
Purpose of the Study:
- To analyze the clinical presentation, treatment modalities, and outcomes of infantile hepatic hemangioendothelioma.
- To evaluate the efficacy of different therapeutic interventions, including medical and surgical options.
- To emphasize the importance of a tailored, multidisciplinary approach in managing IHEH.
Main Methods:
- Retrospective analysis of eight patients diagnosed with infantile hepatic hemangioendothelioma.
- Review of patient demographics, clinical symptoms, tumor characteristics, and treatment protocols.
- Assessment of treatment response and patient survival rates.
Main Results:
- The median age at diagnosis was 24 days, with a female predominance (5/3 ratio).
- Common symptoms included abdominal distention and respiratory distress; four patients had cutaneous hemangiomas.
- Systemic prednisolone was effective in five of six patients; interferon-alpha showed good response with manageable side effects. Overall survival was 87%.
Conclusions:
- Treatment strategies for IHEH should be individualized based on tumor burden, symptoms, and associated complications.
- A multidisciplinary team approach, involving pediatricians, surgeons, oncologists, and radiologists, is essential for optimal patient management.
- While medical therapies like corticosteroids and interferon-alpha are effective, surgical intervention carries significant risks.
Background/Aims:
Hepatic hemangioendotheliomas are rare tumors in childhood. We report our 10-years' experience in a tertiary health center.
Methods:
This retrospective analysis included eight patients with infantile hepatic hemangioendothelioma.
Results:
The median age at diagnosis was 24 days (age range: 1 to 70 days) and the female/male ratio was 5/3. The main symptoms were abdominal distention and respiratory distress. Cutaneous hemangiomas were present in four cases. Three infants had Kasabach-Merritt syndrome. Four cases had single hepatic tumors while the others had multiple. The tumor size ranged from 2 cm to 10 cm in diameter. These lesions were located equally in the right and left hepatic lobes, and three babies had bilobar involvement. Most of the multifocal hepatic tumors were associated with skin hemangiomas. Treatment options were assessed individually. Systemic prednisolone therapy (2 mg/kg/d) was commenced in six patients. Five patients responded to corticosteroids. One boy with Kasabach-Merritt syndrome did not respond to this therapy. Interferon-alpha (1 million units (MU)/m2/day) was started, and the daily dose of the drug was increased up to 10 MU/m2, administered 3 times per week, until clinical improvement was achieved. The response was very good and we observed only constitutional adverse symptoms. Two cases were operated; one died from intraoperative bleeding. Other patients were alive and well for 11 to 66 months. Overall survival was 87% in our series.
Conclusions:
The treatment approaches depend on the center's experience. A multidisciplinary approach is required for the best treatment option.
Related Concept Videos
01:28Esophageal Varices-II: Clinical Features and Management
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol abuse, or...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...