Table 2.
Oncologic outcomes
| Number of patients (%) | |
|---|---|
| Response after first line of treatment | |
| CR | 71(80) |
| PR | 4(4) |
| SD/PD | 5(6) |
| NA | 9(10) |
| First Relapse/Progression | 35(40) |
| Intracranial | 19(21) |
| Medullar | 15(17) |
| Metastatic outside CNS | 9(10) |
| Bone | 6(7) |
| Bone marrow | 1(1) |
| Lymph nodes | 1(1) |
| Peritoneum | 1(1) |
| Number of relapses. [range] | [1-9] |
| Time to first progression. median [range months] | 30 [3-148] |
| Type of strategies at progressions | |
| Surgery | 10(11) |
| RT, Radiosurgery or RT/Surgery | 8 (9) |
| CT/RT | 3(3) |
| CT ± target therapy alone | 50(56) |
| CT/surgery | 5(6) |
| Carboplatin-based | 20(22) |
| Temozolomide-based | 15(17) |
| Cisplatin-based | 10(11) |
| Bevacizumab-based | 8(9) |
| SHH driver-based | 7(8) |
| Others | 7(8) |
CR, complete response; CT, chemotherapy; NA, non-available; PD, progression disease; PR, Partial response; RT: radiotherapy; SD, stable disease; SHH, sonic hedgehog.
Carboplatin-based: Carboplatin/etoposide (CARBO/VP-16). Carboplatin/etoposide with dose reduction craniospinal irradiation protocol regimen (RSMA protocol). Carboplatin/etoposide with thiotepa and temozolomide (CARBO/VP-16-TTP-TD). Ifosfamide, carboplatin and etoposide (IFO/CARBO/VP-16). Carboplatin/etoposide high dose chemotherapy by thiotepa, stem cell transplant, radiotherapy, and maintenance temozolomide (PNET HR5). Temozolomide-based: Temozolomide alone. Temozolomide/topotecan (TOTEM). Temozolomide/irinotecan (TEMIRI). Temozolomide/etoposide (TD/VP-16). Cisplatin-based: Cisplatin/etoposide. Cisplatin, lomustine, vincristine. (PACKER). Cisplatin, vincristine and fotemustine (POF). Cisplatin, vincristine, lomustine, procarbazine, hydroxyurea, aracytin, cyclophosphamide in 1-day chemotherapy regimen (8 in 1). Bevacizumab-based: Bevacizumab, thalidomide, celecoxib, fenofibric acid, etoposide, cyclophosphamide, cytarabine (MEMMAT). Bevacizumab/irinotecan. SHH driver-based: Vismodegib alone. Vismodegib/temozolomide. Others: Procarbazine, belustine, vincristine (PCV). Caryolysine, vincristine and procarbazine (PPOM). Cyclophosphamide.