paediatrics Brussels 17

J Neurooncol (2006)

Table 2 Patients who failed the primary treatment

Patient Age

Tumor location

Primary treatment

Site of recurrence

Time to recurrence (months)

Salvage therapy/outcome (survival duration after salvage)

(years)

STR a

1

22

L1–4

Distant

78

Surgery, CSI, and CHT/Dead-unknown (27 years)

and local RT

2

14

T6–L4

GTR

Local

15

Surgery/NED (10 years)

and local RT

3

27

L1–3

STR

Distant

11

Surgery, whole spine RT, and CHT/AWD (21 years)

and local RT

4 5

31 20

L3–4 L1–3

STR

Local Local

20 40

Surgery and local RT/NED (19 years)

Bx

Surgery/NED (5 years)

and local RT

6 7 8 9

16 28 19 35 33 60 26

L2 L2

GTR STR

Local Local

5

Surgery and RT/NED (10 years) Surgery and RT/NED (10 years) CSI and CHT/AWD (11 years)

55 11

T5–7/T12–L1 GTR T12–L3/L5–S1 GTR

Distant and local

Distant

8

CSI/NED (7 years) RT/NED (6 years) CHT/DOD (2.5 years) RT/AWD (4 years)

10 11 12

S1–5

GTR

Local Local Local

70

T12–L4 T9–L2

STR and CSI

5

GTR

23

a STR = Subtotal resection; RT = Radiotherapy; CSI = Craniospinal irradiation; CHT = Chemotherapy; GTR = Gross total resection; NED = No evidence of disease; AWD = Alive with disease; BX = Biopsy; DOD = Dead of disease

Discussion

In the current study, no significant effect of the different initial surgical extents on OS or PFS was found. However, in patients who had either GTR or STR, adjuvant radiotherpy showed better LC rates (90% for both) at 10 years, compared with GTR or STR alone (58% and 0%, respectively). This result was comparable to that reported by Waldron et al. [18], who likewise observed no influence of the extent of resection. These authors also showed no recurrences in 11 patients with low-grade tumors treated with GTR and adjuvant RT; similarly, in our study, only 1 (10%) of 10 patients who had GTR plus adjuvant RT had disease recurrence. Furthermore, Shaw et al. [25] observed recurrences in three (44%) of seven patients treated with GTR and postoperative RT. In a Mayo Clinic series on MPEs, only 7 (16%) of the 45 patients who underwent GTR had recurrences [14]. Several authors have demonstrated the importance of the extent of surgical resection in determining recurrence patterns and do not support the use of adjuvant RT after GTR in spinal ependymoma [20, 23, 28, 29]. In our experience, MPEs can be technically tricky to resect completely because of the anatomic complexity of the cauda equina. Intraoperative ultrasonography can be quite helpful in disclosing occult foci hiding behind the cauda equina [19]. The main asset to a safe and effective operation in this disease, however, re- mains an experienced, persistent, technically skillful surgeon [30]. Recently, the use of monitoring tech- niques, such as the recording of somatosensory evoked potentials, and the earlier diagnosis made possible by CT and MRI have made tumors (including MPEs)

In our study, patients treated for histologically proven MPE had excellent long-term survival rates: the 10-year OS rate was 97%, and the 10-year PFS and LC rates were 62% and 72%, respectively. Most recur- rences occurred within the primary site of disease. No recurrences occurred outside the neural axis. Most importantly, our data support the notion that patients who initially receive adjuvant radiotherapy to maxi- mize local control have improved PFS rates. Our results compare favorably with those of other series in the literature that have focused on spinal ependymomas, including MPEs. For example, 10-year survival rates of 75% to 100% have been reported by other authors [9, 14, 16, 18, 25–27] (Table 3).

10 12 14 16 18

B C D E

0 2 4 6 8

Number of Patients

Pre-radiotherapy

Last Follow-up

Fig. 4 Neurologic function according to Frankel classification

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