Neuroblastoma: a 1990 overview

Abstract

Neuroblastoma is the most common solid tumor in infancy. This tumor is of neural crest origin and occurs in the neck (3%), mediastinum (20%), pelvis [3], adrenal medulla (50%), and paraspinal sympathetic ganglia (24%). The tumor may produce catecholamines, vasoactive intestinal polypeptide, and ferritin. More than 50% of cases have metastases at the time of diagnosis. Radiologic (CT scan, scintigraphy) examinations, bone-marrow aspirate and biochemical testing (urinary VMA) often pinpoint the diagnosis and permit initiation of therapy. Treatment is based on the extent of disease according to staging criteria. Stages I and II reflect localized disease that is resectable and frequently requires no other therapy. Stage III and stage IV disease represent advanced and metastatic disease, respectively, and require aggresiive combined treatment, including multiagent chemotheraphy, total-body irradiation, and rescue with bone-marrow transplantation using autologous narrow purged of tumor cells with monoclonal antibodies. Delayed primary and second-look resection of tumor is an integral part of this therapy. Survival is based on age and stage at the time of therapy. Infants under 1 year of age and those with stages I, II, and IV-S disease have an improved prognosis. Aneuploid flow cytometry, low serum ferritin and NSE levels, few or no copies of the N-myc oncogene, favorable histology and primary tumors affecting the neck, pelvis and mediastinum have an improved prognosis. The survival in 266 cases was 44.5%.