Abstract

Soft tissue sarcomas, as exemplified by the treatment of rhabdomyosarcoma in children, now can be eradicated effectively in the majority of patients. In order to achieve this success, a combined treatment approach in a cancer treatment center utilizing a close coordination of surgery, radiation therapy, chemotherapy and supportive therapy is necessary. VAC chemotherapy with vincristine, actinomycin D and cyclophosphamide has been the combination reported with the most successful results to date. At least some of each of the less common types of soft tissue sarcoma in children have also shown responsiveness to this therapy. Current recommended therapy for osteosarcoma involves surgical removal of the primary, whenever possible, with amputation of the entire involved bone. Intensive chemotherapy to eradicate micrometastases is of value in achieving increased chances for long-term survival. Metastatic lesions should be approached aggressively with combinations of surgery, radiation therapy and/or chemotherapy, with a resultant increased chance for long-term survival. The vast majority of children with Wilms' tumor now can be treated successfully. Proper diagnostic studies and treatment under the direction of a multidisciplinary cancer treatment team allow the child with Wilms' tumor an excellent chance for long-term survival. Utilization of proper surgical technic, radiation therapy dependent on the extent of tumor and age of the patient and chemotherapy all contribute to the potential successful treatment. In Stage I disease, radiation therapy may not be necessary. Actinomycin D in combination with vincristine is more effective than either drug alone. Adriamycin also is an effective drug in the treatment of Wilms' tumor. Neuroblastoma remains the only major solid tumor in children in which significant advances in treatment have not been accomplished. Total surgical removal remains the treatment of choice whenever possible. Children under the age of 1 year frequently have achieved long-term survival without evidence of disease despite the presence of metastatic disease and without intensive therapy. Intensive combination therapy with radiation and chemotherapy is being utilized in an attempt to find a more effective way to eradicate this tumor. Retinoblastoma is one of the most treatable of the malignant tumors of childhood. Early diagnosis and treatment by an experienced treatment team can produce long-term disease-free survival without significant loss of vision in the majority of patients treated. Current advances in intensive combination therapy for systemic tumors may offer possibilities for long-term control of patients with extensive or metastatic retinoblastoma.

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