A shunt or other cerebrospinal fluid diversion procedure may be needed.
In selected patients in whom a portion of the tumor has been resected, the patient may be observed without further disease-directed treatment, particularly if the pace of tumor regrowth is anticipated to be very slow. Approximately 50% of patients with less-than-gross total resection may have disease that remains progression-free at 5 to 8 years, supporting the observation strategy in selected patients.
Radiation therapy is usually reserved until progressive disease is documented [16,21] and may be further delayed through the use of chemotherapy, a strategy that is commonly employed in young children.[22,23] For children with low-grade gliomas for whom radiation therapy is indicated, conformal radiation therapy, intensity-modulated radiation therapy, or stereotactic radiation therapy approaches appear effective and offer the potential for reducing the acute and long-term toxicities associated with these modalities.; [Level of evidence: 3iDiii] Care must be taken in separating radiation-induced imaging changes from disease progression during the first year after radiation, especially in patients with pilocytic astrocytomas.[26,27,28]; [Level of evidence: 2A]; [Level of evidence: 2C]; [Level of evidence: 3iiiDi]; [Level of evidence: 3iiiDii]; [15,33][Level of evidence: 3iiiDiii]
Radiation therapy results in long-term disease control for most children with chiasmatic and posterior pathway chiasmatic gliomas, but may also result in substantial intellectual and endocrinologic sequelae, cerebrovascular damage, and possibly an increased risk of secondary tumors.[8,34,35,36]; [Level of evidence: 2C]
Radiation therapy and alkylating agents are used as a last resort for patients with NF1, given the theoretically heightened risk of inducing neurologic toxic effects and second malignancy in this population. Children with NF1 may be at higher risk for radiation-associated secondary tumors and morbidity due to vascular changes.
An alternative to immediate radiation therapy is subtotal surgical resection, but it is unclear how many patients will have stable disease and for how long.
Given the side effects associated with radiation therapy, postoperative chemotherapy may be initially recommended.