April 28, 2019
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Radiation after surgery increases survival in pediatric ependymoma

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Administering radiation therapy immediately after surgery to children aged younger than 3 years with ependymoma extended EFS to levels seen in older children, according to results of a phase 2 trial published Journal of Clinical Oncology.

“Historically, children [younger than] the age of 3 [years] with ependymoma have a worse prognosis than older children,” Thomas E. Merchant, DO, PhD, chair of the department of radiation oncology at St. Jude Children’s Research Hospital, said in a press release. “However, results from this clinical trial show that even in young children, survival can be improved when radiation is administered immediately after surgery.”

Merchant and colleagues analyzed 356 pediatric patients (age range, 1-21 years; 57.9% male) with classic ependymoma (n = 215) or anaplastic ependymoma (n = 141) that had not been previously treated with chemotherapy or radiotherapy.

Tumor location was infratentorial in 258 patients, supratentorial in 96 patients and transtentorial in two patients. Patients with classic supratentorial ependymoma underwent observation following gross-total resection. Patients who underwent subtotal resection received chemotherapy, a second surgery and immediate postoperative conformal radiation therapy (CRT).

Those who underwent near-total or gross-total resection received immediate postoperative CRT, with a cumulative CRT dose of 59.3 Gy. Patients who underwent a gross-total resection and were younger than 18 months received a dose of 54 Gy.

Median follow-up was 7.89 years (range, 0.09-11).

Results showed 5-year EFS rates of 61.4% (95% CI, 34.5-89.6) in the observation group, 37.2% (95% CI, 24.8-49.6) in the subtotal resection group and 68.5% in the near-total/gross-total resection group.

Five-year EFS rates varied significantly based on tumor grade (P = .0044), but appeared similar based on age, location, RELA fusion status, or posterior fossa A/posterior fossa B grouping. Patients aged younger than vs. older than age 3 years did not show significantly different rates of 5-year EFS (62.9% vs. 70.5%) and OS (87.4% vs. 85.8%).

In comparison, the Pediatric Oncology Group study POG 9233 — which evaluated standard or dose-intensified chemotherapy for children with ependymoma, with radiation therapy deferred to time of progression — showed a 5-year EFS of 24.4% and 5-year OS of 42.7%.

Patients with infratentorial without 1q gain had a higher EFS rate (82.8%; 95% CI, 74.4-91.2) than patients with 1q gain (47.4%; 95% CI, 26-68.8; P = .0013).

“These results are already shifting the standard of care for patients with ependymoma because the clinical trial used standard conformal radiation, which is widely available,” Maryam Fouladi, MD, chair of the Children’s Oncology Group central nervous system committee, said in a press release. – by John DeRosier

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Disclosures: Merchant reports travel expenses from Philips Healthcare. Please see the study for all other authors’ relevant financial disclosures.