Resection of the primary tumor is appropriate for children with stage IV-S neuroblastoma: an analysis of 37 patients.

Current recommendations for treatment of children with IV-S neuroblastoma (NB) indicate that "supportive care is the cornerstone of therapy" and resection of the primary tumor is not mandated. The presentation and clinical management of 37 IV-S NB patients from three pediatric oncology centers were retrospectively reviewed. The 22 boys and 15 girls presented at an average age of 107 days (range, 4 to 616 days). Primary tumor sites were identified in the adrenal gland in 19 children, the thorax in 5, the retroperitoneum in 4, and 1 each in the pelvis and kidney. At the time of presentation, metastatic disease was documented in the liver (27), bone marrow (19), skin (7), and regional lymph nodes (7). Massive hepatomegaly (below the umbilicus) was demonstrated in 18 patients. Twenty-eight children (76%) have survived on average 90 months following diagnosis. Eight patients (22%) died; six of disease and two of complications of therapy (mean, 9.6 months). One child was lost to follow-up. Only 3 of the 37 patients (8%) were managed solely by supportive care and all are alive. Extirpation of the primary tumor was accomplished in 24 individuals (65%). Twenty patients underwent excision at diagnosis and four had delayed resection with few postoperative complications and no deaths related to resection. One death resulted from progression of disease in a child who had tumor removal. Chemotherapy as a single treatment or in combination with radiation was used in 10 children with three children surviving (30%).(ABSTRACT TRUNCATED AT 250 WORDS)