paediatrics Brussels 17

I. J. Radiation Oncology ● Biology ● Physics

290

Volume 46, Number 2, 2000

Table 4. Univariate analyses of the correlation between selected parameters and estimated progression-free survival rate in 55 children with anaplastic ependymomas

Table 3. Patient characteristics according to treatment volume

CSI ( n 5 40)

Local irradiation ( n 5 13)

Variable

3-yr Progression- free survival rate (%)

The median single dose was 1.6 Gy (range, 1.5–1.6 Gy). The median boost dose was 20.0 Gy with a median single dose of 2.0 Gy (range, 1.5–2.0 Gy). The cumulative median dose to the posterior fossa was 55.2 Gy (range, 42–56 Gy); 10% of the children had a cumulative dose of less than 50 Gy; 2.5% of the children had a dose of more than 55.2 Gy. Eleven children with supratentorial primary tumors un- derwent craniospinal irradiation because the tumor had in- filtrated the ventricular system, and 1 child underwent this treatment because of dissemination of the disease. The parents of 2 children refused irradiation. Both children had localized supratentorial primary tumors and underwent complete resection plus sandwich chemotherapy, according to the design of the HIT 91 trial. Table 3 shows patient characteristics according to the volume of radiotherapy. Survival Follow-up ranged from 5 to 106 months (median, 38 months). The 3-year-estimated overall survival rate and progression-free survival rate were 75.6% and 59.7%, re- spectively (Fig. 2). The pathologic findings were not eval- uated by the review committee in 20 patients included in the analysis. We estimated the overall and progression-free survival rates in the reviewed children separately and found Median age (range) 7.2 yr (3.6–8.8 yr) 5.3 yr (3.0–16.6 yr) Tumor site Infratentorial 28 1 Supratentorial 12 12 Resection Complete 20 6 Incomplete 20 7 M-Stage M0 35 13 M1–3 5 0 CSI 5 craniospinal irradiation. yr 5 years.

No. of patients ( n 5 55)

Parameter

95% CI

p Value

Age (years) 1–6

26 29 28 27 29 26 28 27 15 40 40 13 5 50

66.2 46.8–85.6 0.63

. 6

58.4 37.6–79.2

Sex

Male

67.5 42.9–80.9 0.35

Female

57.1 36.1–78.1

Tumor site

Infratentorial Supratentorial

53.1 33.1–73.1 0.71

72.4 52.8–92.0

Metastases Yes

0

0–35.1 0.0001*

No

69.0 51.3–80.3

Resection

Complete Incomplete

83.3 68.2–98.4 0.0043*

38.5 16.2–60.8

Chemotherapy Maintenance

61.9 35.6–88.2 0.25

Sandwich

63.7 47.6–79.8

Treatment volume CSI

56.3 39.8–72.8 0.44

Involved field

92.3 77.8–100

CSI 5 Irradiation of craniospinal axis. * Significant.

no difference (76% and 62%, respectively). Sixteen (29%) children died of recurrent disease. One child died of severe septic complications after salvage surgery. No other deaths occurred. Patterns of failure At the time of the last follow-up, 30 children were free of disease, and 25 patients showed progression. Disease re- curred at the primary tumor site only in 20 patients (36.4% of all patients, 80% of failures). Disease disseminated within the CNS in 3 (5.5%) children: 1 case was meningeal and intracranial, 1 case was intracranial, and 1 case was in the thoracic spinal canal. Two children (3.6%) suffered from combined distant (1, intracranial; 1, not specified) and local failure. Impact of clinical variables on outcome Table 4 summarizes the correlations between the clinical variables and both the estimated overall and progression- free survival rates. Of the factors associated with both the overall and progression-free survival rates, leptomeningeal dissemination or solid metastases at the time of diagnosis were found to be significant. All 5 children with positive CSF cytology findings ( n 5 4) or spinal metastases ( n 5 2) died within 2 years of surgery. In contrast, children with localized tumors achieved a progression-free survival rate of 65.8% at 3 years (Fig. 3). Neither age, sex, or tumor site showed an impact on treatment outcome. The relationship

Fig. 2. Kaplan-Meier plots of the estimated overall and progres- sion-free survival rates of 55 children.