Benign cerebellar astrocytomas in children: a clinical picture and results of treatment

Telegina A.A., Akopova R.Yu.

(Polenov Research Neurosurgical Institute, Saint Petersburg, Russia)

The analysis of 85 patients, treated in the Department of Children's Neurosurgery of the Polenov Research Neurosurgical Institute in 1981-2000, was carried out with the purpose of studying peculiarities of a clinical course of cerebellar astocytomas and factors, which effect prognosis.

One of the most frequent symptoms was headache, watched in 82 patients. Every third child suffered from it since the disease onset. But it was not of a hypertensive character and resembled headache typical of muscle tension or vascular disorders. It appeared during different hours of a day (often in the day-time or in the evening) and was a result of fatigue. This headache was not accompanied by nausea or vomiting. It was pressing and throbbing and ceased after rest or taking analgesics or vascular drugs.

Vomiting was observed in 80 patients. Isolated vomiting was seen in 19 patients; it was the first symptom of the disease in 12 of them.

A pathologic position of a head was watched in a half of all cases. The forced position, present in every seventh child, was the first and only manifestation of cerebellar astrocytoma during a period of time, ranging from 3 weeks up to 4 years. Disorders of cerebellum functions, marked to a greater or lesser degree, were typical of all cases. Cerebellar gait, as the first symptom of the disease, was observed in 12 patients.

The first symptoms differed in their rate, depending on a child's age. Headache was the first symptom in 83% of children, who were more than 7 years old; but it was watched only in 38% of younger patients. As for the rest 62% of the latter age group, the first symptoms included vomiting, a forced position of a head and cerebellar gait. Their rate was identical. Disorders of functions of the sixth and eighth nerves, manifesting themselves just at the diseases onset, were watched in single cases.

During the first visit to a doctor a tumor was suspected only in 27% of patients; thus, misdiagnosis was a ground for treatment of the rest 73% of children, which lasted some months and even years (up to 13). It should be emphasized, that treatment with vascular drugs, nootropes, diuretics, vitamins and physiotherapy resulted in temporary disappearance (6 months -10 years) or less marked manifestation of symptoms in 41 patients.

Signs of hydrocephalus and increased intracranial pressure were present in 84 patients. Changes of an eye fundus, manifesting themselves in the form of engorged optic disks, were watched in 63 cases. Secondary atrophy of the optic nerve was diagnosed in 8 children.

A marked failure of sight (from 0.09 up to amaurosis) was typical of every seventh child. Stem symptoms were observed practically in all cases. Signs of midbrain, pons and medulla oblongata lesions were present in 73%, 81.2% and 58.8% of patients respectively.

Total removal was achieved more often, when astrocytoma was localized within the cerebellum (68.3%). The same index in tumor growth into the forth ventricle or brain stem was equal to 27.3%. Early postoperative mortality was 8.2%. On admission a state of children, who died, was estimated as severe or extremely severe. Tumor grew into the forth ventricle or brain stem in all of them. There was no early postoperative mortality in a group of patients with astrocytomas, localized within the cerebellum. Catamnesis (1 year-27 years) is known in 56 patients; 8 children died of tumor; terms of survival varied from 10 months up to11.5 years. An index of a total period of survival was 85.7%.

Recurrences were observed in 30% of children. They took place during the first 5 years after an operation in the majority of cases; they developed later (7-14.5 years) only in 1/7 of patients. Irradiation and chemotherapy did not improve prognosis in benign cerebellar astrocytomas, which were not removed completely. Recurrences after subtotal or partial neoplasm resection were diagnosed in 58.8% of patients, subject to an operation only, and in 52.7% of cases, treated with combined methods.

There was no statistically reliable difference in the rate of recurrences and mortality, depending on age (older or younger than 7 years old), macroscopic findings (cystic or solid tumor), a histobiological type (diffuse or nodular astrocytoma), presence of endothelial proliferation, nuclear or cellular polymorphism, mitosis and necrosis. The only significant factor, correlating with relapses and death, was a degree of neoplasm removal. The rate of mortality and recurrences in a remote period was 4.3% and 8.3% respectively in total removal; these figures were equal to 21.2% and 60.6% in incomplete resection.

Almost a half of patients demonstrate no pathology in neurologic examination and live a full-value life. Mild neurologic disorders are watched in 27.7% of cases, but they do not affect working ability. Moderate or marked disorders of neurologic or psychic functions are present in 15% of patients; they have a negative impact on life quality. Gross neurologic disturbances are observed in 6.4% of children, who need constant assistance.