Resection of primary tumor in stage 4S neuroblastoma: a second study by the Italian Neuroblastoma Group
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ORIGINAL ARTICLE
Resection of primary tumor in stage 4S neuroblastoma: a second study by the Italian Neuroblastoma Group Stefano Avanzini1 · Isabella Buffoni2,7 · Anna Rita Gigliotti3 · Stefano Parodi3 · Irene Paraboschi1,7 · Alessandro Inserra4 · Patrizia Dall’Igna5 · Anna Maria Fagnani6 · Giuseppe Martucciello1,7 · Mario Lima8 · Umberto Caccioppoli9 · Alberto Garaventa2 · Massimo Conte2 · Claudio Granata10 · Angela Rita Sementa11 · Elisa Tirtei12 · Giovanni Erminio3 · Bruno De Bernardi2 Accepted: 11 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose To clarify the role of primary tumor resection in stage 4S neuroblastoma. Methods We investigated a cohort of 172 infants diagnosed with stage 4S neuroblastoma between 1994 and 2013. Of 160 evaluable patients, 62 underwent upfront resection of the primary tumor and 98 did not. Results Five-year progression-free and overall survival were significantly better in those who had undergone upfront surgery (83.6% vs 64.2% and 96.8% vs 85.7%, respectively). One post-operative death and four non-fatal complications occurred in the resection group. Three patients who had not undergone resection died of chemotherapy-related toxicity. Thirteen patients underwent late surgery to remove a residual tumor, without complications: all but one alive. Outcomes were better in patients diagnosed from 2000 onwards. Conclusion Infants diagnosed with stage 4S neuroblastoma who underwent upfront tumor resection had a better outcome. However, this result cannot be definitely attributed to surgery, since these patients were selected on the basis of their favorable presenting features. Although the question of whether to operate or not at disease onset is still unsolved, this study confirms the importance of obtaining enough adequate tumor tissue to enable histological and biological studies to properly address treatment, to achieve the best possible outcome. Keywords Neuroblastoma · Stage 4S · Surgery · Primary tumor resection Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00383-020-04766-1) contains supplementary material, which is available to authorized users. * Stefano Avanzini [email protected] 1
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Pediatric Surgery Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Commenda 10, 20122 Milan, Italy
Pediatric Surgery Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147 Genoa, Italy
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Oncology Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147 Genoa, Italy
DINOGMI, University of Genoa, Largo Paolo Daneo 3, 16132 Genoa, Italy
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Epidemiology and Biostatistics Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147 Genoa, Italy
Pediatric Surgery Unit, University Hospital Authority St. Orsola-Malpighi Policlinic, Via Massarenti 11, 40138 Bologna, Italy
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Division of General and Thoracic Surgery, IRCCS Ospedale Pediatrico Bambino Gesù, Piazza S. Onofrio 4, 00165 Rome, Italy
Pediatric Surgery Unit, Santobono-
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