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. 2021 Jan;37(1):37-47.
doi: 10.1007/s00383-020-04766-1. Epub 2020 Oct 29.

Resection of primary tumor in stage 4S neuroblastoma: a second study by the Italian Neuroblastoma Group

Stefano Avanzini  1 , Isabella Buffoni  2   3 , Anna Rita Gigliotti  4 , Stefano Parodi  4 , Irene Paraboschi  5   3 , Alessandro Inserra  6 , Patrizia Dall'Igna  7 , Anna Maria Fagnani  8 , Giuseppe Martucciello  5   3 , Mario Lima  9 , Umberto Caccioppoli  10 , Alberto Garaventa  2 , Massimo Conte  2 , Claudio Granata  11 , Angela Rita Sementa  12 , Elisa Tirtei  13 , Giovanni Erminio  4 , Bruno De Bernardi  2
Affiliations

Resection of primary tumor in stage 4S neuroblastoma: a second study by the Italian Neuroblastoma Group

Stefano Avanzini et al. Pediatr Surg Int. 2021 Jan.

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; Primary tumor resection; Stage 4S; Surgery.

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