We report 80 supratentorial lobar ependymomas, including 46 recurrences. All underwent operation. In the total collection 34.5% survived for more than 5 years. The behaviour of children and adults was nearly identical. Based on a histological grading into three steps of malignancy (E1, E2, E3), the ependymomas in low grade 1 showed a 5 years' survival time of 41.5%. Compared to a 28.5% survival in group E2 at the same time. Results in group E3, which histologically resembled malignant glioma or glioblastoma, are not relevant because of the small number of cases. More than 50% of the E1 recurrences showed transformation to E2. No transformation into a lower grade of malignancy has been observed.
In a series of 310 cases the role of the factors age, sex, location of the tumour (midline or lateral), and medulloblastoma type (classic or desmoplastic) were analysed with regard to the prognosis. The influence of therapy is not considered because of the inhomogeneity of the material and the view nowadays that treatment is insufficient. The occurrence of lateral (or hemispheric) medulloblastomas increases in the group of the 11-15 years old patients. The proportion of the desmoplastic type is greater in adults than in children, but they are distributed almost equally medially and laterally. There seems to be no definite correlation between sex and the survival time. The mean survival time increases with age. With regard to the histological subclassification into children and adults, the mean survival times are nearly identical. The only obvious factor with a decisive influence on the prognosis seems to be the time of appearance of the tumour. Additionally, the data support the conclusion that a desmoplastic medulloblastoma should be considered as a histological variant without clinical relevance.
Out of 29 supratentorial ependymomas in children under 10 years of age, operated on between 1951 and 1989, 18 were situated in the hemispheres and 11 in the midline. 15 of the 18 hemispheric tumours, but only 4 of the 11 intra- or paraventricular ependymomas allowed complete removal. The operative mortality within the observation period of nearly 40 years was 27% for tumours in the midline and 11% for those in the hemispheres. The grade of malignancy rose with increasing distance from the ventricular level. 5-year survival without recurrence was 75% in grade 2 and 31% in grade 3 ependymomas. The total rate of recurrence was 58%. New tumour growth can be delayed by postoperative irradiation, at least in grade 2 ependymomas. It can be prevented, if at all, only by total resection of the primary tumour.
Intracranial ependymomas tend to spread along on the liquor pathways and thus to seed subarachnoid metastatic implants. According to autopsy data, spinal seeding can be expected in 25% of cases subsequent to surgery of the primary tumor. Analysis of four of our own cases (out of 125 primary intracranial ependymomas) together with those described in the literature suggests clinical evidence of seeding in 75% of patients. 47 of 75 metastases originated from malignant infratentorial ependymomas. Malignant ependymomas metastasize earlier than benign ones. They are characterized by disseminated seeding of tumor implants along the entire spinal subarachnoid space. The median survival time after diagnosis of seeding was 6 months. 80% of all patients died within the first 12 months following diagnosis. The necessity of prophylactic spinal radiation therapy in the course of the initial treatment of intracranial ependymomas has not yet been proven.
According to the grading of brain tumors as proposed by the WHO in 1976, out of 128 ependymomas 83 tumors could be classified as grade II and 38 as grade III Only seven subependymomas were benign and could be assigned to grade I. In contrast to most series known from the literature, 73 ependymomas were located above the tentorium and only 55 in the posterior cranial fossa. The grade of malignancy rised with an increased distance from the ventricular level. Macroscopically complete exstirpations were usually possible in hemispheric ependymomas, whereas tumors arising from the floor of the fourth ventricle often allowed only a partial removal. The operative mortality in the infratentorial group was more than twice as that in the supratentorial group. Postoperative survival was predominantly dependent on the histologic grade of malignancy. The five year survival rate without recurrence was 57.4% in grade II ependymomas as compared to 24.1% in grade III ependymomas. It could be improved by postoperative radiation therapy in both groups of malignancy. The almost identical longterm results indicate that even in less malignant ependymomas new tumor growth will occur later on.
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