paediatrics Brussels 17

between the tumor site and the progression-free survival

rate is shown in Fig. 4.

Impact of treatment variables on outcome

The treatment-related variables associated with progres-

sion-free survival are also summarized in Table 4. The

patients with macroscopically complete resection (

28)

fared significantly better, with an estimated overall survival

rate of 91.5% at 3 years, than those who underwent incom-

plete resection (

27), with an estimated overall survival

rate of 56.1% (

0.046). The estimated progression-free

survival was also significantly better for children with com-

pletely resected tumors (Fig. 5).

The maintenance chemotherapy or sandwich chemother-

apy did not alter the prognosis. Specifically, children who

were treated for disease in the neuraxis with an additional

boost to the tumor site showed no difference in outcome

compared with the children who were treated with irradia-

tion at the tumor site only. Of the children who did not

receive any radiotherapy, 1 is alive after 5 years (her tumor

specimen was not reviewed) and 1 died of local and distant

disease progression after 1.5 years.

The distribution of risk factors in patients with supraten-

torial tumors given radiotherapy to the craniospinal axis or

the tumor region is shown in Table 5, and the survival rate

distribution is shown in Fig. 6. Because of very uniformly

administered radiotherapy, it is difficult to draw conclu-

sions; however, we did not find an impact of fraction size or

total dose on the survival rate.

DISCUSSION

Ependymomas account for 3% to 4% of childhood can-

cers (1). There is little information on the outcome of

different treatments for ependymomas and still no consen-

sus on the optimal therapy. Most studies have investigated

low- and high-grade ependymomas, despite several reports

about a worse outcome in patients with anaplastic ependy-

momas (12–15). Only WHO grade III ependymomas were

included in the two German brain tumor trials described

here.

The outcome in patients with ependymomas remains

suboptimal, although the survival rates have increased from

24% (16) to 60% and 70% (12, 17). Regardless of the

therapy administered in the patients in the present study,

those with unfavorable factors, such as incomplete resection

and tumor dissemination, had a poor outcome, with progres-

sion-free survival at 3 years of 38% and 0%, respectively.

Disease recurrence at the primary site is still the main

obstacle to cure, occurring in 88% of all cases of progres-

sion in our study. Similar rates have been observed in other

Fig. 3. Relationship between initial dissemination and the esti-

mated progression-free survival rate.

Fig. 4. Relationship between tumor site and estimated progression-

free survival rate.

Fig. 5. Relationship between the extent of resection and the esti-

mated progression-free survival rate. compl. resec, complete re-

section; incompl. resec., incomplete resection.

Table 5. Characteristics of 24 supratentorial ependymomas

according to treatment volume*

Variable

CSI

Local field

Total number* of tumors

Resection

Complete

Incomplete

M stage

M1–3

* Of the 26 children with supratentorial ependymomas, 2 were

not irradiated.

291

Anaplastic ependymomas in childhood

●

B. T

IMMERMANN

et al.