Spinal Ganglioneuroma: A Systematic Review of the Literature

World Neurosurg. 2023 Dec:180:163-168.e7. doi: 10.1016/j.wneu.2023.08.057. Epub 2023 Sep 1.

Abstract

Objective: Spinal ganglioneuromas (GNs) are rare benign tumors that often manifest as symptoms related to the compression of neural elements. The preferred treatment for affected patients is surgical resection, which typically improves symptoms and accompanies a low likelihood of tumor recurrence. We conducted a systematic review of reports of GNs involving the spinal cord and nerve roots, examining their clinical presentation, surgical management, and outcomes.

Methods: Using the keywords "ganglioneuroma" and "spinal," we conducted a systematic database review of MEDLINE (PubMed), Scopus, and Embase, querying studies reporting cases of spinal GNs. Patients' demographics, location of the tumors, clinical features, and surgical outcomes were extracted from eligible articles.

Results: A total of 93 spinal GN cases in 52 case reports/series met our criteria. Data analysis revealed a general male predominance, though thoracic spinal GNs were seen more in females. The mean age of patients with cervical, thoracic, thoracolumbar, and lumbar spinal GNs were 41.28, 27.65, 15.61, and 38.73 years, respectively. Multiple-level GNs were mostly seen in male patients or individuals with neurofibromatosis type 1. In all but 1 case, recurrence and reoperation were not reported in the short-term (months) and long-term (2-10 years) follow-up.

Conclusions: We found unique epidemiologic characteristics for patients with GNs of different spinal regions. The treatment of choice is achieving gross total resection, but given the eloquency of the lesions, achieving decompression via subtotal resection can also be associated with improved outcomes. To date, no global postoperative surveillance protocol exists, considering the low recurrence rate and relevant cost-benefit ratios.

Keywords: Ganglioneuroma; Review; Spinal ganglioneuroma; Tumors.

Publication types

  • Systematic Review
  • Review

MeSH terms

  • Female
  • Ganglioneuroma* / pathology
  • Ganglioneuroma* / surgery
  • Humans
  • Male
  • Neoplasm Recurrence, Local / epidemiology
  • Neoplasm Recurrence, Local / surgery
  • Neurofibromatosis 1* / surgery
  • Neurosurgical Procedures
  • Spinal Cord Neoplasms* / pathology