Do infants with stage IV-S neuroblastoma need treatment?

Abstract

The study reports the results of treatment in 31 infants under age 12 months with stage IV-S neuroblastoma diagnosed between 1976 and 1979. All had small or undetected primary tumours associated with disease in one or more of the following sites: liver, skin, or marrow. The primary site was left adrenal (in 16), and other areas included paravertebral, mediastinum, and pelvis (in 5); in 3 the primary site could not be found. Distant disease was found in the liver (in 29), marrow (in 16), and skin (in 8). Other sites affected were pancreas, pleura, peritoneum, and regional nodes. Treatment varied according to the clinical course of the disease; most patients had very little. In 19 primary tumour was resected, in 21 the liver was irradiated with a median dose of 450 rad, and 15 received chemotherapy in courses varying between 1 month and 1 year. Nine patients had resection of the primary tumour as their only treatment and all survive; a total of 16 patients had sites of disease which regressed spontaneously. Four of 31 patients died in the first 2 months despite vigorous measures, all from some complication of the disease or its treatment. The projected 2-year survival rate is 87%. Children with this 'special' pattern of widespread neuroblastoma fare well with little or no treatment unless early complications develop. In this study none died of late progression of their disease.