Management of pediatric CNS meningiomas: CCHE-57357 experience in 39 cases

Childs Nerv Syst. 2019 Aug;35(8):1323-1331. doi: 10.1007/s00381-019-04156-6. Epub 2019 May 24.

Abstract

Purpose: Our aim is to present our experience in the management of pediatric meningiomas in the largest referral center in Egypt for pediatric tumors focusing on variables including clinical picture, anatomic location, histopathology, treatment strategies, and outcome together with their possible correlation to prognosis.

Methods: We retrospectively reviewed the medical records of 39 pediatric patients who were treated for CNS meningiomas in Children's Cancer Hospital-Egypt (CCHE-57357) 2007-2017.

Results: The prevalence of pediatric meningioma was 1.42%. Four cases had type 2 neurofibromatosis (NFII). The mean age was 8.19 years. The presence of NFII was associated with challenging multiple lesions, older age of presentation and poorer prognosis and functional outcome. Convexity was the commonest location. Gross total resection (GTR) was achieved in 28 cases, subtotal resection (STR) in 8 cases, and biopsy was decided in 3 patients. Histopathological examination revealed WHO grade I in 16 patients and higher grades in 23 patients (59%). The 5-year overall survival (OS) rate was 87.8% while the 5-years event-free survival (EFS) rate was 85.6%. Tumor location, histopathology, and clinical presentation were not statistically correlated to prognosis.

Conclusions: Pediatric CNS meningiomas are uncommon pediatric tumors but of an aggressive clinical and pathological behaviors as compared to adult meningiomas. The presence of NFII is associated with a poorer prognosis and functional outcomes. Although being challenging, the maximum and safe surgical excision should be exercised even in recurrent cases in order to achieve the best outcome. Adjuvant radiotherapy provides good tumor control for inoperable residual atypical or anaplastic meningiomas.

Keywords: Aggressive meningiomas; Pediatric meningioma; Prognostic factors; Type 2 Neurofibromatosis.

MeSH terms

  • Child
  • Child, Preschool
  • Egypt
  • Female
  • Humans
  • Male
  • Meningeal Neoplasms / genetics
  • Meningeal Neoplasms / pathology*
  • Meningeal Neoplasms / therapy*
  • Meningioma / genetics
  • Meningioma / pathology*
  • Meningioma / therapy*
  • Neurofibromatosis 2 / complications
  • Neurosurgical Procedures / methods
  • Radiotherapy, Adjuvant / methods