Preirradiation chemotherapy

One study demonstrated a benefit of preirradiation chemotherapy in children with near-total resection (>90% resection), with outcomes similar to those for children achieving a gross-total resection followed by radiation therapy.[28]

The COG ACNS0121 (NCT00027846) trial included two cycles of preirradiation chemotherapy for children with residual disease after up-front surgery (n = 64).[8][Level of evidence B4]

The COG ACNS0121 (NCT00027846) trial included two cycles of preirradiation chemotherapy for children with residual disease after up-front surgery (n = 64).[8][Level of evidence B4]

Second-look surgery occurred in 39% of patients (n = 25) (gross-total resection, 56%; near-total resection, 20%; subtotal resection, 24%).

The COG ACNS0121 (NCT00027846) trial included two cycles of preirradiation chemotherapy for children with residual disease after up-front surgery (n = 64).[8][Level of evidence B4]

For patients who underwent second-look surgery, the 5-year EFS rate was 50.5%, compared with 28.5% for patients who did not undergo second surgery (P = .12).

A multi-institutional trial for children younger than 3 years used preirradiation chemotherapy, followed by conformal radiation once the child was older than 12 months, followed by maintenance chemotherapy.[29][Level of evidence B4]

A multi-institutional trial for children younger than 3 years used preirradiation chemotherapy, followed by conformal radiation once the child was older than 12 months, followed by maintenance chemotherapy.[29][Level of evidence B4]

Fifty-four patients were enrolled, and 54% of patients (n = 29) underwent a gross-total resection at diagnosis.

A multi-institutional trial for children younger than 3 years used preirradiation chemotherapy, followed by conformal radiation once the child was older than 12 months, followed by maintenance chemotherapy.[29][Level of evidence B4]

Of the remaining 25 patients, 60% (n = 15) underwent a second-look surgery after chemotherapy, with 80% of patients achieving a gross-total resection.

A multi-institutional trial for children younger than 3 years used preirradiation chemotherapy, followed by conformal radiation once the child was older than 12 months, followed by maintenance chemotherapy.[29][Level of evidence B4]

At the time of radiation therapy, 76% of patients had a gross-total resection, 13% of patients had a near-total resection, and 11% of patients had a subtotal resection.

A multi-institutional trial for children younger than 3 years used preirradiation chemotherapy, followed by conformal radiation once the child was older than 12 months, followed by maintenance chemotherapy.[29][Level of evidence B4]

PFS (but not OS) was better for patients who underwent a gross-total resection or near-total resection before radiation therapy than it was for patients who underwent a subtotal resection (4-year PFS rate, 79% for gross-total resection/near-total resection vs. 41.7% for subtotal resection) (P = .024).

The SIOP Ependymoma I study enrolled 74 patients, 41 of whom had a subtotal resection after initial surgical management. The protocol specified that these patients were to receive up to four cycles of preirradiation vincristine, etoposide, and cyclophosphamide (VEC). Of the 41 patients, 10 did not receive the protocol-specified VEC therapy, and 3 patients opted for no further therapy and did not receive radiation therapy.[24][Level of evidence B4]

The SIOP Ependymoma I study enrolled 74 patients, 41 of whom had a subtotal resection after initial surgical management. The protocol specified that these patients were to receive up to four cycles of preirradiation vincristine, etoposide, and cyclophosphamide (VEC). Of the 41 patients, 10 did not receive the protocol-specified VEC therapy, and 3 patients opted for no further therapy and did not receive radiation therapy.[24][Level of evidence B4]

Of the 29 patients who received VEC, the combined complete response and partial response rate was 65%, which exceeded the prespecified 45% response rate threshold.

The SIOP Ependymoma I study enrolled 74 patients, 41 of whom had a subtotal resection after initial surgical management. The protocol specified that these patients were to receive up to four cycles of preirradiation vincristine, etoposide, and cyclophosphamide (VEC). Of the 41 patients, 10 did not receive the protocol-specified VEC therapy, and 3 patients opted for no further therapy and did not receive radiation therapy.[24][Level of evidence B4]

Eight of 29 patients had progressive disease at the completion of VEC chemotherapy. The 5-year and 10-year EFS rates were 34%. The 5-year OS rate was 60%, and the 10-year OS rate was 54%.

The SIOP Ependymoma I study enrolled 74 patients, 41 of whom had a subtotal resection after initial surgical management. The protocol specified that these patients were to receive up to four cycles of preirradiation vincristine, etoposide, and cyclophosphamide (VEC). Of the 41 patients, 10 did not receive the protocol-specified VEC therapy, and 3 patients opted for no further therapy and did not receive radiation therapy.[24][Level of evidence B4]

The study demonstrated chemoresponsiveness in most patients. However, the small number of patients precluded a determination of the added benefit of preirradiation VEC in either facilitating a subsequent gross-total resection or in a survival benefit, compared with patients with a subtotal resection who opted not to receive VEC before radiation therapy.