Management of Brainstem Gliomas in Children

Initial Management at Presentation

• Categorization of tumor: Since treatment varies with tumor type, the first step in management of a brainstem glioma is to categorize it as a focal, exophytic, diffuse, or cervicomedullary tumor.
• Focal, exophytic, and cervicomedullary tumors: These tumors can be managed initially with surgery. They all share the feature that treatment tolerance is a function of the clinical state of the patient. Therapy is best tolerated by a patient with minimal or no symptoms or signs of neurological dysfunction. Therefore, treatment is best initiated at the time of the tumor’s discovery.
• Diffuse intrinsic pontine glioma: Patients with diffuse intrinsic pontine gliomas should be evaluated by the oncology team for radiation therapy, with or without adjuvant chemotherapy.

Adjunctive Therapies

• Diffuse intrinsic pontine glioma: Patients with diffuse intrinsic pontine gliomas may receive chemotherapy before, concomitant with, or after radiation therapy. To date, however, chemotherapy has no proven efficacy.

Follow-up

• Schedule dictated by expectations of tumor behavior: Beyond the standard postoperative visits, follow-up is dictated by the tumor pathology (benign versus malignant), extent of resection, and postoperative neurological status.
• Benign tumors: Benign tumors require close imaging follow-up initially after surgical resection.  Eventually, if MRI scans show that the tumor is stable, imaging can be obtained annually in the absence of new symptoms.
• Malignant tumors: Malignant tumors should be followed more closely in a multidisciplinary fashion with early involvement by neuro-oncology. These patients will be seen frequently by oncologists as adjuvant therapy is administered.