Neuroblastoma stage 4S: Tumor regression rate and risk factors of progressive disease

• Published in: Pediatric blood & cancer 2020-04, Vol.67 (4), p.e28061-n/a
• Main Authors: Tas, Michelle L., Nagtegaal, Michelle, Kraal, Kathelijne C.J.M., Tytgat, Godelieve A.M., Abeling, Nico G.G.M., Koster, Jan, Pluijm, Saskia M.F., Zwaan, C. Michel, Keizer, Bart, Molenaar, Jan J., Noesel, Max M.
• Format: Article
• Language: English
• Subjects: Catecholamines; Compression; Excretion; Hematology; Liver; Metastases; Neuroblastoma; Oncology; Patients; Pediatrics; Risk factors; spontaneous neoplasm regression; stage 4S; Tumors
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.28061

Background The clinical course of neuroblastoma stage 4S or MS is characterized by a high rate of spontaneous tumor regression and favorable outcome. However, the clinical course and rate of the regression are poorly understood. Methods A retrospective cohort study was performed, including all patients with stage 4S neuroblastoma without MYCN amplification, from two Dutch centers between 1972 and 2012. We investigated the clinical characteristics, the biochemical activity reflected in urinary catecholamine excretion, and radiological imaging to describe the kinetics of tumor regression, therapy response and outcome. Results The cohort of 31 patients reached a 10‐year overall survival of 84% ± 7% (median follow‐up 16 years; range, 3.3‐39). During the regressive phase, liver size normalized in 91% of the patients and catecholamine excretion in 83%, both after a median of two months (liver size: range, 0‐131; catecholamines: range, 0‐158). The primary tumors completely regressed in 69% after 13 months (range, 6‐73), and the liver architecture normalized in 52% after 15 months (range, 5‐131). Antitumor treatment was given in 52% of the patients. Interestingly, regression rates were similar for treated and untreated patients. Four of seven patients