Infantile hepatic hemangioendothelioma: clinical presentation and treatment.
OBJECTIVE
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.
Sevinir
Betül
B
Department of Pediatric Oncology, Uludağ University, School of Medicine, Bursa. bsevinir@yahoo.com
Ozkan
Tanju B
TB
D000005:Abdomen; D000410:Alanine Transaminase / Q000378:metabolism; D001219:Aspartate Aminotransferases / Q000378:metabolism; D004108:Dilatation, Pathologic / Q000209:etiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D006391:Hemangioma / Q000175:diagnosis; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D016898:Interferon-alpha / Q000627:therapeutic use; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D009378:Neoplasms, Multiple Primary; D011239:Prednisolone / Q000627:therapeutic use; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis; D014421:Turkey / Q000453:epidemiology
D005938:Glucocorticoids; D007155:Immunologic Factors; D016898:Interferon-alpha; D011239:Prednisolone; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase