Diagnosis and management of pediatric brain-stem gliomas

The authors reviewed the cases of 49 children, ranging in age from 9 months to 15 years, who were diagnosed by computerized tomography (CT) as having brain-stem glioma. Four distinct groups of brain-stem gliomas were identified based on CT scan characteristics: Group I included isodense contrast-enhancing tumors that were dorsally exophytic into the fourth ventricle; Group II(a) included hypodense nonenhancing intrinsic tumors of the brain stem; Group II(b) included intrinsic tumors of the brain stem with hyperdense exophytic components extending ventrally and laterally into the cerebellopontine and prepontine cisterns; Group III included intrinsic cystic tumors with contrast-enhancing capsules; and Group IV included focally intrinsic tumors of the brain stem that were isodense and enhanced brightly on administration of contrast medium. The clinical presentation, efficacy of surgical intervention, pathology, and prognosis of these tumors were correlated within these groupings. Eleven patients had Group I tumors, all of which were surgically resected; 10 of the 11 lesions were proven to be low-grade gliomas. These patients had an excellent prognosis; 10 of the 11 survived, with a mean follow-up period of 4.5 years. There were 18 patients with Group II(a) tumors; although tumor biopsy was attempted on eight of these, pathological diagnosis at the time of surgery was made in only one case. These patients did poorly; the mean survival time was 6.2 months. The seven Group II(b) tumor patients demonstrated a similarly poor prognosis: all of them died within 23 months of diagnosis, with a mean survival time of 12 months. Only two of six patients undergoing biopsy had sufficient tissue for histological verification. Three of the four patients with Group III tumors died; their mean survival time was 11.5 months. Successful histological examination was carried out in all four cases. The nine Group IV tumor patients did reasonably well; seven of these patients remain alive, with a mean follow-up period of 2.3 years. Histological diagnosis was obtained in three of the seven patients who were explored in this group. This classification system has proven to be of value in determining prognosis and efficacy of surgical intervention.