Biology and therapy of solid tumors in childhood

In children malignancies have characteristics distinct from tumors occurring in adults. For instance, the reported incidence is substantially lower, varying from 90 to 170 cases per 1,000,000 child years. Long-term event-free survival rates above 80% are noted in several tumors. Biology is essentially different; in pediatrics the number of mutations is limited and they are linked with specific malignancies, whereas in adults an accumulation of multiple mutations is assumed. Hereditary factors are noted in several neoplasm's. Although the linkage of syndromes with specific tumors is known for a prolonged time, currently the link with minor congenital anomalies in specific tumor types gets more prominent. Environmental factors related with an increased or a decreased risk of cancer are noted.In this review an update is given on tumor biology and treatment for several tumors, i.e. neuroblastoma, Ewing sarcoma, osteosarcoma, nephroblastoma, rhabdomyosarcoma, hepatoblastoma and retinoblastoma. Among the aspects dealt with are the occurrence of spontaneous remissions, I-meta-iodobenzylguanidine (MIBG) imaging and treatment, apoptosis induction, antisense therapy, immunotherapy, tyrosin kinase inhibition, stem cell transplantation, suicide gene therapy and anti-angiogenic therapy.