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← Childhood Ependymoma

HEALTH PROFESSIONAL · SOURCE READING

Radiation Therapy and/or Chemotherapy

Source: Childhood Ependymoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: January 6, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.

Context: Treatment of Recurrent Childhood Ependymoma

Patients with recurrent ependymomas should be considered for treatment with the following modalities:[5][Level of evidence C1]

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Focal retreatment with various radiation modalities, including stereotactic radiosurgery,[6-8][Level of evidence C1]; [9-11][Level of evidence C2]; [12] intensity-modulated photon therapy, and proton therapy.[13]; [14][Level of evidence C1]

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Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

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Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

The median duration of OS was 75.1 months, and the median freedom from progression was 27.3 months.

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

The 1-, 2-, and 5-year estimates of OS rates were 95.5%, 74.9%, and 57.3%, respectively.

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

Among the 46 patients who received focal reirradiation for local failure, 13 had local failures, and 11 had distant-only failures.

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

Among the ten patients who received craniospinal irradiation for local failure, six had local failures, and none had distant-only failures.

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

Patients with distant-only failure who were treated with craniospinal irradiation had improved OS compared with individuals with local failure who were treated with focal radiation therapy (hazard ratio [HR], 0.37; 95% confidence interval [CI], 0.16–0.87).

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

The 10-year cumulative incidence of greater than grade 3 radiation necrosis after repeat radiation therapy was 7.9%.

Craniospinal irradiation for both local and distant (spinal) recurrence could be considered.[15] A study of 101 reirradiated patients conducted at St. Jude Children's Research Hospital observed the following results:[13][Level of evidence C2]

Gain of chromosome 1q was associated with poorer OS (HR, 3.5; 95% CI, 1.1–10.6) for patients with distant failure (but not local failure) after initial radiation therapy. Other variables associated with reduced OS and freedom from progression included male sex, anaplastic histology at recurrence, and a short interval from initial radiation therapy to recurrence.

In a study of 31 children with locally recurrent ependymomas, patients received local (conformal) radiation at recurrence. They received various fractionation schemes using radiation doses similar to those used at initial diagnosis.[16]

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In a study of 31 children with locally recurrent ependymomas, patients received local (conformal) radiation at recurrence. They received various fractionation schemes using radiation doses similar to those used at initial diagnosis.[16]

The median local recurrence-free survival was 31 months (range, 2–63 months), and the median OS was 34 months (range, 3–63 months).

In a study of 31 children with locally recurrent ependymomas, patients received local (conformal) radiation at recurrence. They received various fractionation schemes using radiation doses similar to those used at initial diagnosis.[16]

Patients who underwent surgery first had significantly higher survival rates than did patients who received reirradiation only.

In contrast, a study of 53 patients with recurrent ependymomas treated with radiation therapy after surgery showed no advantage with the addition of radiation therapy after a gross-total or near-total resection.[4][Level of evidence B4]

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In contrast, a study of 53 patients with recurrent ependymomas treated with radiation therapy after surgery showed no advantage with the addition of radiation therapy after a gross-total or near-total resection.[4][Level of evidence B4]

There was an improvement in the 5-year OS rate (22% vs. 7%) for patients treated with radiation therapy who had less than gross-total or near-total resections.

Three, and even four, courses of radiation therapy for patients with recurrences can prolong survival with acceptable minimal toxicity.[13][Level of evidence C2]

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Active anticancer agents, including cyclophosphamide, cisplatin, carboplatin, lomustine, and etoposide, have been used in the recurrence setting. While older single-agent studies have demonstrated chemoresponsiveness with these agents, response is rarely durable.[17,18]

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Regardless of treatment strategy, the prognosis for patients with recurrence is poor.[1] Entry into studies of novel therapeutic approaches should be considered.

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Publication references

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Preserved source evidence · Not medical advice