Stefano Avanzini1, Isabella Buffoni2,3, Anna Rita Gigliotti4, Stefano Parodi4, Irene Paraboschi5,3, Alessandro Inserra6, Patrizia Dall'Igna7, Anna Maria Fagnani8, Giuseppe Martucciello5,3, Mario Lima9, Umberto Caccioppoli10, Alberto Garaventa2, Massimo Conte2, Claudio Granata11, Angela Rita Sementa12, Elisa Tirtei13, Giovanni Erminio4, Bruno De Bernardi2. 1. Pediatric Surgery Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. stefanoavanzini@gaslini.org. 2. Oncology Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. 3. DINOGMI, University of Genoa, Largo Paolo Daneo 3, 16132, Genoa, Italy. 4. Epidemiology and Biostatistics Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. 5. Pediatric Surgery Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. 6. Division of General and Thoracic Surgery, IRCCS Ospedale Pediatrico Bambino Gesù, Piazza S. Onofrio 4, 00165, Rome, Italy. 7. Pediatric Surgery Unit, Department of Women's and Children's Health, University of Padua, Via Giustiniani 3, 35128, Padua, Italy. 8. Pediatric Surgery Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 10, 20122, Milan, Italy. 9. Pediatric Surgery Unit, University Hospital Authority St. Orsola-Malpighi Policlinic, Via Massarenti 11, 40138, Bologna, Italy. 10. Pediatric Surgery Unit, Santobono-PausiliponChildren'sHospital, Via della Croce Rossa 8, 80122, Naples, Italy. 11. Radiology Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. 12. Pathology Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy. 13. Division of Pediatric Oncology, Regina Margherita Children's Hospital, Piazza Polonia 94, 10126, Torin, Italy.
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.
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.
Entities:
Keywords:
Neuroblastoma; Primary tumor resection; Stage 4S; Surgery
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