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ACNS0121 showed that focused radiotherapy to the tumour bed straight after surgery cures most children with ependymoma, including those under three who used to be denied radiotherapy, and that children whose tumour could not be fully removed do far worse even with chemotherapy and a second operation.
ACNS0121 was a Children's Oncology Group phase 2 trial of 378 children with newly diagnosed ependymoma, assigned by extent of resection and histology. Children with completely resected, differentiated supratentorial tumours were observed; those with near-total or gross-total resection received immediate post-operative conformal radiotherapy to 59.4 Gy (54 Gy under 18 months); those with subtotal resection received chemotherapy (vincristine, carboplatin, cyclophosphamide, etoposide) before second-look surgery and radiotherapy. The primary endpoint was event-free survival.
Five-year event-free survival was 68.5 percent after immediate conformal radiotherapy, 61.4 percent in the observation group and 37.2 percent after subtotal resection. Outcomes were similar for children under three, tumour grade mattered, and 1q gain in posterior fossa tumours predicted relapse (five-year event-free survival 47.4 against 82.8 percent). The corpus's ependymoma page describes maximal resection followed by conformal or proton radiotherapy to the tumour bed as the ACNS0121 approach.
Numbers are from the trial as recorded here; see the source links in the table below. This is orientation, not medical advice: ask your team how closely the trial population matches you.
378 enrolled.
95% CI 62.8 to 74.2 · 95% CI 34.5 to 89.6 · 95% CI 24.8 to 49.6
Source| Endpoint | Arm | n | Value | HR (95% CI) | p | Source |
|---|---|---|---|---|---|---|
| Event-free survival at 5 years by treatment groupprimary | Near-total or gross-total resection then immediate conformal radiotherapy | - | 68.5% | - | - | link |
| Observation after complete resection of differentiated supratentorial ependymoma | - | 61.4% | ||||
| Subtotal resection: chemotherapy, second surgery, radiotherapy | - | 37.2% | ||||
| Event-free survival at 5 years, infratentorial tumours by 1q status | Without 1q gain | - | 82.8% | - | 0.0013 | link |
| With 1q gain | - | 47.4% |
Shares Brain and spinal cord tumours (all types), Childhood cancers (all types), Children's Oncology Group (COG), Vincristine and the tag soc-trials.
Shares Brain and spinal cord tumours (all types), Childhood cancers (all types), Children's Oncology Group (COG), Vincristine and the tag soc-trials.
Shares Brain and spinal cord tumours (all types), Childhood cancers (all types), Children's Oncology Group (COG), Etoposide and the tag soc-trials.
Shares Childhood cancers (all types), Vincristine, Etoposide, Cyclophosphamide and the tag soc-trials.
Shares Brain and spinal cord tumours (all types), Childhood cancers (all types), Children's Oncology Group (COG), Etoposide and the tag soc-trials.
Shares Childhood cancers (all types), Children's Oncology Group (COG), Vincristine, Etoposide and the tag soc-trials.
Shares Childhood cancers (all types), Children's Oncology Group (COG), Vincristine, Etoposide and the tag soc-trials.
Shares Childhood cancers (all types), Children's Oncology Group (COG), Vincristine, Etoposide and the tag soc-trials.