paediatrics Brussels 17

Bifractionated RT in childhood ependymoma d C. C ONTER et al .

1539

DISCUSSION

Table 2. Pattern of failures

Treatment of EP is one of the most controversial issues in pediatric oncology. The published data in the field have mostly been mono- or oligocentric studies and mainly retro- spective. Few randomized studies have been reported. The prognostic factors and oncologic strategies are a matter of de- bate. CR is usually shown as the main prognostic factor (2, 14, 15) , although some series (6) , including ours, have failed to confirm this finding. In most series, CR was obtained in about one-half of patients, although surgery is generally less successful in patients with infratentorial lesions (14) . Pathologic grading is a matter of major debate. Some series have described it at a major prognostic factor (16–18) , and others have failed to show any difference with grade (9, 19–21) . The results obtained in our series failed to show any difference. Whether this was a result of the low number of patients included or differences in the grading assessment remains to be demonstrated. Recently, discrepancies between histologic assessments have been reported in infants (9, 10) . Whether this applies only to infants or also is true for older children remains unresolved. An international panel of experts is currently reviewing such cases to answer this question.

Local ( n = 7)

Distant ( n = 1)

Distant and local ( n = 3)

Variable

Grade

2 ( n = 6) 3 ( n = 5)

3 4

1 0

2 1

Location

Infratentorial ( n = 10) Supratentorial ( n = 1)

6 1

1 0

3 0

Extent of resection Complete ( n = 7) Partial ( n = 2) Doubtful ( n = 2)

5 0 2

0 1 0

2 1 0

complete remission, 1 had stable disease, and 1 had disease progression. Relapse developed in 11 patients. The rates of relapse were similar between patients with Grade 2 and Grade 3 tumors (6 of 13 vs. 5 of 10, respectively; Table 2 ). The patient whose histologic grade was not assessable did not develop a relapse. One-half of the patients with infratentorial and the one-fourth of patients with supratentorial EP developed a relapse. The rate of relapse was similar among the patients with CR, in- complete resection, or doubtful resection (7 of 16, 2 of 4, and 2 of 4, respectively). Disease recurred once in 8 patients, twice in 2, and five times in 1 patient. The median time to the first relapse was 22 months (range, 4–46). Seven patients had only local failure; one had an isolated distant failure; and three had combined failure ( Table 2 ). Of the 10 local relapses, 9 were infield relapses and 1 occurred outside the radiation field. The treatment of failure was surgery alone in 1 patient, chemotherapy alone in 2, RT alone in 1, surgery and chemo- therapy in 3, surgery, RT, and chemotherapy in 3, and palli- ative treatment in 1 patient. The median survival after relapse was 12.5 months (range, 0–44). Long-term side effects The long-term side effects among the 16 patients alive at completion of the study are reported in Table 3 . Three-fourths of the patients had normal psychomotor development, as as- sessed by the local physician. Of the 16 patients, 9 underwent an IQ test. Wechsler scale III or IV were used, depending on the age of the patient. After a median follow-up of 41 months (range, 7–66), the mean verbal IQ was 93 (range, 54–130), and the mean performance IQ was 88 (range, 42–111). Two patients required placement in a special school, and two were $ 2 years behind at school. One patient had tran- sient growth hormone deficiency requiring growth hormone supplementation with rapid normalization of the size. No other endocrine disorder was reported, but 4 of the 24 patients were in prepuberty stage. Their size was normal (with 1 to +1 standard deviation for age and gender) in all patients. One patient with a supratentorial tumor had a severe decrease of visual acuity secondary to high intracranial pressure with optic atrophy and required adapted schooling.

Table 3. Long-term side effects among 16 living patients

Patients evaluated ( n )

Variable

Sequelae

Auditory

15

Normal

12

Loss <40 dB at 1,000–8,000 Hz on one ear Loss >40 dB at 1,000–8,000 Hz or <40 on both ears Normal school or university (possibly 1 y behind) Normal school but >2 years behind Special school and currently employed Special school and currently unemployed

1

2

Schooling

16

12

2

1

1

Vision

16

Normal Diplopia

9 5 1 1

Mild decrease of visual acuity Severe decrease of visual acuity

Psychomotor development

16

Normal

12

Mild retardation Severe retardation

2 2

Endocrine deficit

16

None

15

Hypothyroidism

0 1

Growth hormone deficiency Premature or delayed puberty

0 *

* Four in prepubertal stage.