Incidence, Treatment, and Survival in Primary Central Nervous System Neuroblastoma

World Neurosurg. 2020 Aug:140:e61-e72. doi: 10.1016/j.wneu.2020.04.145. Epub 2020 Apr 25.


Background: Primary central nervous system neuroblastoma (PCNSN) is a rare disease, and its incidence, treatment modalities, and survival remain poorly understood.

Methods: The SEER (Surveillance Epidemiology and End Results) database was used to identify patients diagnosed with PCNSN from 1973 to 2013. The incidence and survival rates were examined. Clinical features, treatment modalities, and prognosis were also assessed.

Results: A total of 280 patients with PCNSN were identified, with annual age-adjusted incidence being 0.37 per 1,000,000 persons in 1973 and decreasing to 0.12 in 2013. Neuroblastoma (NBL) (ganglioneuroblastoma vs. NBL; odds ratio [OR], 25.01; P = 0.008) and tumor with distant metastasis (OR, 0.17; P = 0.002) were more likely to receive conservative treatment over surgery, whereas older age (OR, 1.02; P = 0.011) and tumors located in the brain (other nervous system vs. brain: OR, 0.31; P = 0.001) increased the likelihood of receiving combined surgery and radiotherapy over surgery alone. In addition, younger age, ganglioneuroblastoma, and surgery treatment were significantly associated with improved outcomes (all P < 0.05). Furthermore, a nomogram model was established to effectively estimate survival for patients with PCNSN.

Conclusions: We updated epidemiologic information of PCNSN and showed that age, histologic type, tumor extension, and surgery were independent prognostic factors. Moreover, treatment modalities of these tumors are influenced by patient and tumor characteristics.

Keywords: Central nervous system; Incidence; Neuroblastoma; SEER; Survival.

MeSH terms

  • Adolescent
  • Adult
  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / mortality*
  • Central Nervous System Neoplasms / therapy
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Incidence
  • Infant
  • Male
  • Neuroblastoma / diagnosis*
  • Neuroblastoma / mortality*
  • Neuroblastoma / therapy
  • Retrospective Studies
  • SEER Program / trends
  • Survival Rate / trends
  • Young Adult