Surgical management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed

Pediatr Surg Int. 2017 Sep;33(9):955-959. doi: 10.1007/s00383-017-4100-9. Epub 2017 Jun 12.

Abstract

Purpose: Clinical researches about the management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed are limited. We report the surgical outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed in a single institution.

Methods: Ganglioneuroma and ganglioneuroblastoma-intermixed diagnosed and resected between May 2009 and May 2015 in a tertiary children's hospital were retrospectively reviewed. Patients' demographic data, INSS stage, surgical complications, residual tumor size and outcomes were collected.

Results: Thirty-four patients were included in the current study. All had localized tumors and were surgically managed. The overall acute complications rates were 8.8% (3/34) and none were fatal. Thirty-three of 34 patients had at least macroscopic tumor resection. Six patients had radiographically detected residual tumor after surgery, 25 none and 3 undocumented. Thirty-three (97.1%) patients were alive during a median follow-up of 36 months (range 1-82). In subgroup analysis, no significant difference regarding surgical complications and survival was found between ganglioneuroma and ganglioneuroblastoma-intermixed. Increased complete resection rates were observed in thoracic tumor compared with abdominal ones (p = 0.03). However, no significant difference (p = 0.089) regarding overall survival was found between patients with residual tumors and those without. Of the six patients with residual tumors, three showed complete resolution, two were unchanged and one died 3 years after initial surgery (the only death in this study).

Conclusion: Ganglioneuroma and ganglioneuroblastoma-intermixed can be safely and effectively resected, the residual tumor seems not to influence overall survival.

Keywords: Ganglioneuroblastoma; Ganglioneuroma; Neuroblastic tumor; Surgery.

MeSH terms

  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / surgery
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Ganglioneuroblastoma* / pathology
  • Ganglioneuroblastoma* / surgery
  • Ganglioneuroma* / pathology
  • Ganglioneuroma* / surgery
  • Humans
  • Infant
  • Male
  • Neoplasm Staging
  • Neoplasms, Multiple Primary* / pathology
  • Neoplasms, Multiple Primary* / surgery
  • Retrospective Studies
  • Thoracic Neoplasms / pathology
  • Thoracic Neoplasms / surgery