Table 1.
EPN-PFB | PF-SE/E | EPN-ZFTA | |
---|---|---|---|
Number, n (%) | 56 (32.9) | 44 (25.9) | 19 (11.2) |
Age, [y] median (range) | 39 (18-66) | 60 (29-81) | 37 (20-63) |
Sex ratio | |||
Male to female | ~1:1 | ~8:1 | ~2:1 |
KPS, n (%) | |||
<70 | 4 (11) | 9 (25) | 1 (12) |
70–80 | 11 (31) | 14 (39) | 2 (25) |
90–100 | 21 (58) | 13 (36) | 5 (63) |
No data | 20 | 8 | 11 |
Extent of surgery, n (%) | |||
Subtotal | 18 (34) | 18 (45) | 2 (13) |
Gross total | 35 (66) | 22 (55) | 13 (87) |
No data | 3 | 4 | 4 |
Adjuvant therapy, n (%) | |||
None | 36 (67) | 35 (81) | 4 (25) |
RT alone | 18 (33) | 8 (19) | 9 (56) |
Chemotherapy alone | 0 (0) | 0 (0) | 0 (0) |
RT + Chemotherapy | 0 (0) | 0 (0) | 3 (19) |
No data | 2 | 1 | 3 |
CNS WHO Grade, n (%) | |||
2 | 49 (88) | 41 (93) | 1 (5) |
3 | 7 (12) | 3 (7) | 18 (95) |
Tumor location, n (%) | |||
Supratentorial | 2 (4) | 2 (5) | 19 (100) |
Infratentorial | 54 (96) | 42 (95) | 0 (0) |
Drop metastases, n (%) | |||
No | 45 (88) | 34 (97) | 15 (88) |
Present at diagnosis | 2 (4) | 0 (0) | 0 (0) |
During disease course | 4 (8) | 1 (3) | 2 (12) |
No data | 5 | 9 | 2 |
Disease progression (local recurrence/ metastases) | |||
Events, n (%) | 21 (38) | 13 (31) | 6 (38) |
No data | 1 | 2 | 3 |
Death, n (%) | |||
Events, n (%) | 7 (13) | 11 (28) | 2 (11) |
Died of disease | 2 (4) | 1 (3) | 2 (11) |
Perioperative complications | 1 (2) | 3 (8) | 0 (0) |
Other reason | 1 (2) | 2 (5) | 0 (0) |
No data | 3 | 5 | 0 |
5y-PFS-rates (%) | 64.5 | 67.4 | 60.3 |
10y-PFS-rates (%) | 41.8 | 45.3 | 60.3 |
5y-OS-rates (%) | 96.0 | 78.3 | 100.0 |
10y-OS-rates (%) | 83.2 | 67.5 | 75.0 |
Abbreviations: EPN-PFB = posterior fossa ependymoma group B, EPN-ZFTA = ZFTA fusion-positive ependymoma, KPS = Karnofsky performance status, OS = overall survival, PFS = progression-free survival, PF-SE/E = posterior fossa subependymoma molecular class with ependymoma histology, RT = radiotherapy.