Table 2.
Patients with WNT-activated tumours (positive nuclear beta-catenin IHC) and relapse
| ID | CTNNB1 mutation analysis | Other risk factors | Relapse site | CCR1 (months) | Relapse treatment | Survival after 1st relapse (months) | Status | |
|---|---|---|---|---|---|---|---|---|
| R1 | R2 (R3) | |||||||
| W1 | Pos | a | PF | 22 | HDSCT (CBDCA, VP-16) | TMZ + IT maphosphamide (HITSKK-92, Mtx/Depocyte) | 42 | DOD |
| W2 | Pos | No | Met (ST) | 40 | No | No (TMZ, VP-16, GEMOX) | 45 | AWT |
| W3 | Pos | a, d | Met (Spinal) | 18 | TMZ | TMZ | 17 | DOD |
| W4 | Neg | a, d, r | Met (CSF, spinal) | 31 | IT VP-16 + surgery + RT | TMZ | 16 | DOD |
| W5 | Neg | No | PF, Met | 53 | Oral VP-16 | N/A | 2 | DOD |
| W6 | No data | d | Met (Spinal) | 54 | Trophosphamide + VP-16 + RT | TMZ + IT VP-16 | 29 | DOD |
| W7 | No data | No | PF | 67 | Surgery + CBDCA, VP-16 + HDSCR (TT, VP-16) | No | 18 | DOD |
| W8 | No data | No | PF, Met (CSF) | 16 | No data | 29 | DOD | |
Four patients had risk factors for poor prognosis at primary diagnosis; a = age >16 years at diagnosis, d = delayed RT start, r = residual (primary) tumour >1.5 cm2. All tumours were of classic histological subtype except case W5 (desmoplastic MB). Relapse treatment at first (R1) and subsequent relapses (R2, R3)
AWT alive with tumour, CBDCA carboplatin, CCR1 time in continuous complete remission after primary diagnosis, DOD dead of disease, GEMOX gemcitabine + oxaliplatin, CSF cerebrospinal fluid, HDSCT high dose chemotherapy with autologous stem cell rescue, IT intrathecal, Met metastatic relapse in CNS (location), Mtx methotrexate, PF posterior fossa, RT radiotherapy, ST supratentorial, TMZ temozolomide, TT thiotepa, VP-16 etoposide