Management of Central Nervous System Tumors Occurring During Infancy

Initial Management at Presentation

  • Physiological stabilization of the infant
  • Diagnostic imaging
  • Delay if probable desmoplastic infantile ganglioglioma: Consider delayed surgical intervention to allow increase in circulating blood volume over time and further maturation of brain before craniotomy.
  • Maximal resection for AT/RTs: A maximal surgical resection, if possible, is the preferred initial treatment of these tumors.
  • Neuroblastomas: Initial management is with chemotherapy unless the disease is causing neurological dysfunction, in which case emergency decompression is usually performed.

Adjunctive Therapies

  • Chemotherapy used with AT/RTs: Surgery is followed by systemic chemotherapy with combination therapy including cisplatin, vincristine, and etoposide. Intrathecal etoposide may prove to be a useful adjunct.
  • Neoadjuvant chemotherapy used with neuroblastomas: Neuroblastomas are often large at presentation, and neoadjuvant chemotherapy is the preferred option, with the possibility of surgical resection for residual disease in groups with a high risk of progression based on residual disease or molecular biological factors (18). Additionally, surgery is used when there is neurological deterioration during treatment (8). Aydin et al. (2) showed no benefit in neurological outcome for patients with laminectomy in addition to systemic chemotherapy compared with those receiving chemotherapy alone.