paediatrics Brussels 17

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M. Reni et al. / Critical Reviews in Oncology/Hematology 63 (2007) 81–89

8. Follow-up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 8.1. Follow up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Acknowledgment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Biographies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89

Abstract

Ependymomas are rare tumours of neuroectodermal origin classified as myxopapillary ependymoma and subependymoma (grade I), ependymoma (grade II) and anaplastic ependymoma (grade III). The more common location is infratentorial (60%). Age <40 years and extent of surgery appear related to better prognosis, while the role of other prognostic factors, such as tumour grade and tumour site are equivocal. This emphasizes the role of surgery as the standard treatment. Postoperative radiotherapy is indicated in high-grade ependymomas, and is recommended in low-grade ependymomas after subtotal or incomplete resection (confirmed by postoperative MR). Deferral of radiotherapy until recurrence may be considered on an individual basis for patients with MR confirmation of a radical resection. Recommended dose to involved fields is 45–54 Gy for low-grade (grade II) and 54–60 Gy for high-grade ependymomas (grade III). There is no proof that postoperative chemotherapy improves the outcome. At recurrence, platinum-, nitrosourea- or temozolomide-based chemotherapy can be administered, although there is no evidence of efficacy. © 2007 Elsevier Ireland Ltd. All rights reserved.

Keywords: Ependymomas; Treatment; Prognosis

1. General information

was 80%, at 5-year 55%. Survival was very poor in infants (24%), then increased with age: in children aged 1–4 years 44%, at 5–9 years 68% and at 10–14 years 75% [6] .

1.1. Definition

Ependymomas are rare tumours of neuroectodermal origin arising from ependymal cells in the obliterated central canal of the spinal cord, the filum terminale, choroid plexus or white matter adjacent to the highly angulated ventricular surface [1] . Additionally, ependymomas can be found in the brain parenchyma as a result of fetal ependymal cell rests migrating from periventricular areas [2] . About 500 new cases of ependymoma are diagnosed in the European Union each year. The annual incidence rate in Europe is around 2 per million [3] . Ependymoma is slightly more frequent in males than females [3] and occurs in all age groups. About 15% of all patients are children of less than 5 years of age [4] . In children and in the countries of Central and South America and Asia, the annual incidence of ependymoma is less than 2 per million [4] . In North America, Oceania and most of Europe, the rate varies between 2 and 4 per million. In Denmark, Sweden, Finland, the former East Germany and Slovenia, the incidence is at least 4 per million. In Europe, 1-year survival for adults diagnosed with ependymoma during 1990–1994 was 82% and 5-year sur- vival 72%, with no difference between men and women [5] . Five-year survival decreased markedly with age from 79% in the youngest (15–45 years), to 44% in the oldest age group (patients 75 years and over) [5] . The 1-year survival for chil- dren with ependymoma in Europe, between 1990 and 1994, 1.2. Incidence 1.3. Survival

1.4. Aetiology and risk factors

Little is known about the aetiology of ependymomas. A possible relationship between the presence of polyomavirus SV40 has been suggested [7,8] , but could not be confirmed by other studies [9,10] . From a recent study [10] over a 55-year period (69.5 million person years), cancer incidence was not shown to be associated with exposure to SV40-contamined poliovirus vaccine. Incidence data on ependymoma were carefully reviewed because of an increase in ependymoma incidence among children aged 0–4 years in exposed periods and compared with the incidence in preceding unexposed periods. However, the incidence of ependymoma was rela- tively low in the years of SV40 contamination, with a highest incidence observed in 1964, when most children were too young to have received the SV40-contaminated vaccine. An increased incidence of ependymomas has also been reported in neurofibromatosis type 2 [11] . An inverse association between maternal consumption of vitamin supplements dur- ing pregnancy and brain tumours has been observed in five out of six studies on this subject [12] , and one of these studies related specifically to the category of ependymomas [13] .

2. Pathology and biology

2.1. Histopathology

According to the World Health Organization (WHO) [14] , ependymal tumours are being classified as WHO grade 1: myxopapillary ependymoma (occurring almost exclusively