Management of Supratentorial Dysembryoplastic Neuroepithelial Tumors in Children

Initial Management at Presentation

  • Watchful waiting: In selected patients presenting with lesions in eloquent brain areas and easily controlled epilepsy, initial watchful waiting may be appropriate.
  • Biopsy only: Stereotactic or focal biopsy can be misleading and is not advocated in the management of these patients (24).
  • Guided resection for seizure management: Depending on the spectrum of the epilepsy and the degree of intractability, the epileptologists have to conduct more or less extensive monitoring.

Adjunctive Therapies

Adjunctive therapies are generally not given before surgery.

Anticonvulsant medications

  • Managed by epileptologist or neurologist: Antiepileptic drugs will be given under the supervision of the pediatric neurologist, who will decide on the drug of first choice and will titrate the optimal dose for the particular child, depending on seizure type, age, weight, blood levels and efficacy of the treatment in an initial phase.

Chemotherapy

  • No indication: There is no role for chemotherapy in the treatment of DNETs.

Radiotherapy

  • Controversial but not an initial treatment: There is no role for radiotherapy at this stage. Only in partially resected cases can irradiation be discussed, but its role is controversial (see below).

Follow-up

  • If initial treatment is observation, schedule scans and visits for management of seizures: Before surgery, the frequency of visits will be guided by the monitoring of the antiepileptic drugs. In cases of DNETs in eloquent areas where initial watchful waiting is advocated, follow-up with MRI should be performed probably in the same way as for low-grade gliomas: every 4 months in the first year and later, every 6 months or in every situation where there is a suspicion of clinical evolution, e.g., a change in seizure frequency or severity.
  • Initial follow-up per center’s seizure management protocol: The initial follow-up will depend mostly on the neurologist/epileptologist, who will see the child to monitor the anticonvulsant medication, adjust the dose, and monitor for side effects. The neurosurgeon should have a short time to discuss the proposed surgery with the child and his or her parents.
  • Subsequent schedule per center’s oncological protocol: The subsequent schedule will be similar for that used by the center for low-grade gliomas unless seizures remain a problem. In that case, the schedule will be dictated by both the epileptic and the oncological needs of the patient.