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Characteristics and management of ganglioneuroma and ganglioneuroblastoma-intermixed in children and adolescents

Pediatric Blood & Cancer Feb 01, 2018

Alexander N, et al. - This retrospective study was designed to compare the natural history, biological and clinical features of ganglioneuroma (GN) and ganglioneuroblastoma-intermixed (GNB-I) managed by surgery or observation to inform management and surveillance. A slow growth rate was noted with GN and GNB-I. Resection of these could be correlated with significant morbidity. For some GN and GNB-I, watch and wait approaches ought to be considered.

Methods

  • Between 1990 and 2014, researchers included patients (n = 67) with histological diagnosis of GN (50/67) and GNB-I (17/67) at the Hospital for Sick Children.
  • They recorded clinical, pathological features, tumor dimensions, and management.

Results

  • Median age was 6 years (1.3–17.8) and maximal tumor diameter was 6.3 cm (1.4–16.9).
  • In this study, 46 patients (69%) had upfront surgery and 21 (31%) were observed, among the 67 patients.
  • Out of the 21 observed patients 4 later underwent resection.
  • Researchers noted post-operative complications in 15 of the 50 (30%) surgical patients.
  • The presence of imaging-defined risk factors associated with complications (P=0.005).
  • Observed patients were found to be older (median 8.4 vs 5.3 years) and diagnosed more recently.
  • In this study, median growth was 0.3 cm/year and 6 of 21 had progressive disease (PD).
  • All patients were alive and pateints with evaluable imaging displayed 27 complete and 10 partial responses, 19 stable and 6 PD, at median follow-up of 2.2 years (0.2–14.3).
  • For 3 cases, pathology classification changed at resection, however, no GN was reclassified to NB.

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