[Malignant tumors of the cerebral hemisphere in children. A series of 64 cases].

BACKGROUND

Cerebral hemisphere tumors in children represent about 15% of all tumors of central nervous system. 30 to 40% of them are malignant and have a poor prognosis.

PATIENTS AND METHODS

The files of 64 children (30 boys and 34 girls, less than 15 years old) treated between 1970 and 1989 for malignant cerebral hemisphere tumor were analysed. There were 27 astrocytomas, 20 ependymomas, 7 oligodendroblastomas, 6 sarcomas, 2 neuroblastomas, 1 medulloepithelioma and 1 teratoma. The tumor was located in the frontal area in 18 patients; it was temporal in 9, parietal in 8 and occipital in 2 patients. The tumor infiltrated 2 or several lobes in 27 children. The tumor was excised in 58 patients (completely in 36). Radiotherapy was given in 42 children; 18 of them also received chemotherapy.

RESULTS

The time between the first clinical manifestation and diagnosis was longer than 6 months in 20% of cases. Symptoms of increased intracranial pressure were present at onset of the disease in 56% of patients and at admission in 73%. Convulsions revealed the tumor in only 30% of cases. Nine children died during the first post-operative month. The actuarial survival rate was 39 +/- 13% at 5 years and 29 +/- 13% at 10 years. This rate was better for ependymomas (48 +/- 23% at 5 years and 41 +/- 23% at 10 years) than for astrocytomas (25 +/- 18% and 13 +/- 16%). Three of the 27 astrocytomas developed in children who had been treated 6 to 7 years earlier for acute lymphoblastic leukemia. One infant developed acute lymphoblastic leukemia 2 years after surgery and chemotherapy for astrocytoma and one patient, treated at 5 months of age for retinoblastoma, developed an astrocytoma 13 years later.

CONCLUSIONS

Increased intracranial pressure is more frequently the first symptom than are convulsions in malignant cerebral hemisphere tumors. The long interval between the first manifestations and diagnosis suggests degeneration of benign tumors in 20% of cases. The development of a second malignant neoplasm is not exceptional in children. The better prognosis for ependymomas than for astrocytomas indicates that cerebral ependymomas should be excised and irradiated regardless of their grade.