Neuroblastoma.
Neuroblastoma, a disease primarily of infants and younger children, is a tumor deriving from sympathetic neuroblasts. The variety of tumor location, growth and biologic characteristics represents the clinical manifestations: the range comprises spontaneous regression up to eminently malignant development with resistance to any therapy. Sometimes the tumor is located in the patient's abdomen and becomes important as a differential diagnosis to the urologist. In recent years some important research work has been done, mainly in the field of molecular biology, supporting the hypothesis of two different types of neuroblastoma. Miscellaneous markers and imaging techniques with varying validity are available, but first of all the proof of elevated catecholamines in serum or urine, or the histology of the tumor, ensures the diagnosis. The international consensus for diagnosis, staging and response to treatment is a step of far-reaching significance. The prognosis for most patients, especially for older patients with metastatic disease, remains poor, despite the combination of chemotherapy, radiation therapy and surgery. Further attempts are being made to identify the disease in a localized or early stage with the introduction of screening programs-whereby the benefit of the method is not statistically proven. Treatment trials focus at the moment on autologous peripheral blood cell and bone marrow transplantation.
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Department of Pediatrics, University of Mainz Medical School, Germany.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D007223:Infant; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis* / Q000628:therapy; D011379:Prognosis