paediatrics Brussels 17

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281

1

Table 1 Characteristics of the 34 children with ependymoma

0.8

Characteristics

No. of patients

(%)

Overall survival

Age (months) Median

0.6

20.5

Range

1.0–33.0

0.4

Sex

Probability

Male

18 16

52.9 47.1

0.2

Female

3-yrs.-pfs = 27.3 % 3-yrs.-os = 55.9 %

Progression free survival

Site

Infratentorial Supratentorial

31

91.2

0

3 0

8.8

0

20

40

60

80

Spinal

0

Survial (months)

Metastases M0/M x

29

85.3

Fig. 3. Kaplan–Meier plots of overall survival and PFS.

M1

3 2

8.8 5.9

M2/3

with an additional boost to the tumor. The median cumulative total dose to the tumor was 54.0 Gy (range, 20.8–56.4 Gy). The median total dose to the neuraxis was 35.2 Gy (range, 24.0–39.6 Gy). The median dose per fraction was 1.8 Gy (range, 1.4–2.2 Gy). In 12 children, radiotherapy was given immediately after completion of chemotherapy without any sign of recurrence or progression of disease. In nine children, radiotherapy was delayed and, administered only in case of recurrence or progression as salvage therapy. Median time interval between surgery and start of irradiation was 11 months (range, 4–34 months). Survival Follow-up for all patients ranged from 7 to 146 months. In survivors, the median time of follow-up was 76.5 months (range, 53–146). For all patients, the 3-year estimated overall survival rate and 3-year PFS rate were 55.9% (confidence interval ( Z CI) 39.2–72.6) and 27.3% (CI 12.1– 42.5), respectively ( Fig. 3 ). For histologically reviewed children ( n Z 17), PFS rates were estimated separately, but no difference could be detected (3-year overall survival and PFS of 58.8% (CI 35.4–82.2) and 25% (CI 3.8–46.2), respectively). Twenty-one children died of recurrent disease. One chemotherapy-related death occurred. There were no other causes of death. For patients who failed, median time to progression was 8 months and median time to death was 29.5 months (range, 7–95 months). Patterns of failure At last follow-up, nine children were free of disease, and 25 children showed progression (73.5%). Nineteen children (76.0%) failed at the tumor site only. Six children (24%) developed dissemination within the CNS (four of them intracranial, one spinal, and one both intracranial and spinal); all of those six patients had local failures also. Late effects At last follow-up, in five survivors information about late toxicity was available. In two children, growth retardation, pituitary insufficiency and need for hormonal replacement were reported. In one other child, retardation in language and mental development was described. One more child had motor deficits in the left upper arm, and one child suffered from cerebellar ataxia.

Resection

Complete Incomplete

18 16

52.9 47.1

Chemotherapy SKK 87

15 19

44.1 55.9

SKK 92

Radiotherapy CSI C boost

11 10 13

32.4 29.4 38.2 35.3 26.5 38.2

Local field

None

Radiotherapy Preventive

12

Salvage

9

None

13

reviewed in 17 children. In 31 children, the tumor site was infratentorial. No patient with spinal ependymoma was included in the study. In 23 patients,CSF cytologic studies for evaluation of leptomeningeal dissemination were available at presen- tation. Three children had positive CSF cytological findings (13%); in 11 children, CSF-samples were not available, but all children underwent craniospinal imaging. Solid metastases to the CNS were found in two children (5.9%). Twenty-nine children (85.3%) did not present with any metastases ( Table 1 ). Surgery All children underwent surgery. Extent of resection was assessed by postoperative CT/MRI, and was considered to be macroscopically complete in 18 children. Chemotherapy In HIT-SKK 87 trial, 15 children and, in HIT-SKK 92, 19 children were treated. All children received adjuvant chemotherapy. Radiotherapy Thirteen children did not receive any radiotherapy (38.2%). Ten children were irradiated at the primary tumor site only. Eleven children received craniospinal irradiation Treatment