Extraventricular neurocytomas: a systematic review of the literature in the pediatric population

Childs Nerv Syst. 2021 Aug;37(8):2465-2474. doi: 10.1007/s00381-021-05257-x. Epub 2021 Jun 17.


Extraventricular neurocytomas (EVNs) are rare neuroepithelial neoplasms of the central nervous system that were first described in 1997. Most studies in patients with EVNs have incorporated mixed age groups. The tumor's clinical behavior specifically in children has not been explored in depth, while a detailed statistical analysis has never been performed in this age group. Hence, we performed a systematic review to address possible prognostic factors and the appropriate management in children with EVNs. Relevant studies were identified by searching the MEDLINE and SCOPUS databases. We included studies concerning patients 18 years of age or younger who were histologically diagnosed with EVNs. A total of 52 studies with 79 patients were included. The mean age of the patients was ~ 10 years with a male predilection (~ 2:1). Most of these tumors were located in the frontal (49%) lobe. We observed that gross total resection of the tumor was significantly lower in cases of atypical EVNs (p < 0.05). Additionally, atypical EVNs were associated with worse overall survival compared to typical EVNs (p = 0.05). Children 4 years of age or under had a worst outcome (p = 0.001). The patient's sex and the extent of the tumor's resection did not appear to affect the prognosis in a statistically significant manner. Contrary to the results of previous studies, the use of adjuvant radiotherapy or chemotherapy for the treatment of EVNs was not associated with better outcomes in the pediatric population. Thus, a less aggressive management of children with EVNs compared to the adult population is suggested.

Keywords: Extraventricular neurocytomas; Management; Pediatric neurocytomas; Survival analysis.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Brain Neoplasms* / therapy
  • Child
  • Humans
  • Male
  • Neurocytoma* / therapy
  • Prognosis
  • Radiotherapy, Adjuvant
  • Time