Introduction: Currently, few prospective guidelines exist for the surveillance of children with low-risk neuroblastic tumors (LRNBTs), including ganglioneuroma or ganglioneuroblastoma intermixed. This study aims to describe our institutional approach to LRNBT surveillance following surgical resection or nonoperative management. We hypothesize that length of surveillance can be reduced due to low recurrence risk.
Methods: We conducted a retrospective review of patients diagnosed with LRNBT at our institution between January 1, 2010, and December 31, 2022. The majority of patients were followed in our multidisciplinary surgical oncology clinic. We summarized relevant patient data including follow-up imaging modalities and recurrences.
Results: Thirty-eight patients met the inclusion criteria at a median age of diagnosis of 9 ys (interquartile range [IQR]: 5, 12.75). Thirty-seven patients underwent surgical resection, with tumors located in the posterior mediastinum (n = 18), retroperitoneum (n = 14), presacral space (n = 3), pelvis (n = 1), or combined thoracic or retroperitoneal location (n = 1). One patient underwent nonoperative management for a tumor located in the retroperitoneum. Histopathological results demonstrated 18 cases of ganglioneuroma and 20 cases of ganglioneuroblastoma intermixed. The median duration of postoperative follow-up was 27 mos (IQR: 14, 51) and the median clinical visits per year was 2.6 [IQR: 1.5, 3.4). Surveillance imaging was performed at a median frequency of 2.4 times per year (IQR: 1.6, 3.1). Chest radiographs were the most common modality used for thoracic locations (34% of all imaging) and ultrasound was used most commonly for abdominal location (24% of all imaging). During surveillance, one recurrent or residual ganglioneuroma was identified on magnetic resonance imaging at 6.7 mos from presacral resection. This patient underwent repeat surgical resection, and subsequent surveillance demonstrated no further recurrences.
Conclusions: Recurrence was low in this cohort of LRNBT patients. Optimal surveillance strategies for this low-risk pathology would minimize radiation exposure and burden of health-care visits to patients. Further multi-institutional studies are warranted to determine the best imaging modality, as well as the frequency and duration of follow-up visits for LRNBT patients. Future studies should evaluate the acceptability of short-term surveillance for those who achieve complete resection.
Keywords: Ganglioneuroblastoma; Ganglioneuroma; Surveillance.
Copyright © 2024 Elsevier Inc. All rights reserved.