Background: Hepatic vascular tumors (HVT) are the most common benign liver tumors present in infancy and childhood commonly associated with high output cardiac failure. Pediatric HVT usually are divided into infantile hemangioendothelioma (IHE), cavernous hemangioma (HC), and arteriovenous malformations (AVM). The aim of this study was to analyze clinical and histologic features of pediatric HVT in relation to treatment strategies.
Procedure: During last 12 years, 17 children have been treated because of HVT. The diagnosis of HVT was established in all on the basis of clinical and imaging data. A retrospective analysis of clinical records and histopathology divided 17 into: Group 1, neonates; and Group 2, infants and older children.
Results: Radiologic imaging revealed the vascular nature of the tumors in all patients. All nine from Group 1 were symptomatic from AVMs and seven were operated upon. Only one of eight children from Group 2 presented symptoms of AVM requiring surgery; four of five in this group had surgery because of the risk of malignancy. Within Group 1, a mixture of proliferating IHE with microscopic features of AVM was found in most. In three neonates with HVT immunologic and clinical features of cytomegalovirus (CMV) hepatitis were noted. In two Group 2 patients, pure HC was present and in another, the diagnosis of angiosarcoma was established after biopsy of a peritoneal metastasis.
Conclusions: HVT in children demonstrate internal morphologic heterogeneity and an age-related behavior of the disease. We also confirm the proliferative nature of all hemangioendotheliomas (HEs) in children. Further studies on the tumorigenesis of these lesions are needed.
Copyright 2002 Wiley-Liss, Inc.