Radiation therapy

In one single-institution study, 74 patients aged 1 to 21 years were treated with conformal radiation therapy immediately after surgery.[17]

In one single-institution study, 74 patients aged 1 to 21 years were treated with conformal radiation therapy immediately after surgery.[17]

The 3-year progression-free survival (PFS) rate was 77.6% (± 5.8%).

In an expansion of the above series, 107 of 153 patients received conformal radiation therapy immediately after up-front resection.[5][Level of evidence C1]

In an expansion of the above series, 107 of 153 patients received conformal radiation therapy immediately after up-front resection.[5][Level of evidence C1]

The 7-year EFS rate was 76.9% (± 13.5%).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4]

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4]

Stratum 1: Patients with completely resected differentiated histology supratentorial ependymomas who were treated without radiation therapy.

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 1: Patients with completely resected differentiated histology supratentorial ependymomas who were treated without radiation therapy.

The 5-year PFS rate was 61.4% (95% confidence interval, 34.5%–89.6%), with no deaths at 7 years, although only 11 patients were enrolled in this stratum.

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4]

Stratum 2: Patients with subtotally resected ependymomas (both supratentorial and infratentorial) with more than 5 mm residual disease. Treatment consisted of two cycles of chemotherapy followed by second-look surgery and conformal radiation therapy to the tumor bed (adding a 1-cm target clinical volume). Radiation doses were 54 Gy for patients aged 12 to 18 months and 59.4 Gy for patients older than 18 months.

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 2: Patients with subtotally resected ependymomas (both supratentorial and infratentorial) with more than 5 mm residual disease. Treatment consisted of two cycles of chemotherapy followed by second-look surgery and conformal radiation therapy to the tumor bed (adding a 1-cm target clinical volume). Radiation doses were 54 Gy for patients aged 12 to 18 months and 59.4 Gy for patients older than 18 months.

The 5-year PFS rate was 25% for patients in whom a second surgery was not feasible, and 50% for patients in whom a second surgery resulted in a gross-total resection.

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4]

Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

The 5-year PFS rate was 68.5% (range, 62.8%–74.2%).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

Posterior fossa A ependymoma (PF-EPN-A), 1q balanced (without 1q gain): The 5-year PFS rate was 81.5% (range, 71.5%–91.5%).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

PF-EPN-A, 1q gain: The 5-year PFS rate was 35.7% (range, 12.8%–58.6%).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

For patients with PF-EPN-A, distant failure was more common in patients with 1q gain than in patients with 1q balanced (without 1q gain).

A COG prospective study (ACNS0121 [NCT00027846]) enrolled 356 patients between the ages of 1 and 21 years with newly diagnosed ependymoma into four strata.[8][Level of evidence B4] › Stratum 3 and stratum 4: Patients with near-total resection (stratum 3) and gross-total resection (stratum 4). Patients aged 12 to 18 months received postoperative radiation therapy doses of 54 Gy, and patients older than 18 months received doses of 59.4 Gy (adding a 1-cm target clinical volume).

Supratentorial ependymomas: 30 of 39 patients with supratentorial ependymomas who were tested harbored ZFTA fusions, 23 of whom were in stratums 3 and 4. There was no significant difference in survival. The 5-year OS rates exceeded 80%.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23]

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23]

In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

For the intracranial cohort (n = 145), the 7-year EFS rate was 63.4%, the OS rate was 82.6%, and the local control rate was 76.1%.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

Fifty-one patients experienced a tumor recurrence: 26 patients (51%) had local failures, 19 patients (37.3%) had distant failures, and 6 patients (11.8%) had synchronous failures.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

Of the 150 patients, 116 (77.3%) underwent gross-total resection, 5 (3.3%) underwent near-total resection, and 29 (19.3%) underwent subtotal resection.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

For the intracranial cohort, the 7-year EFS rate was 70.3% for patients who underwent a gross-total resection or near-total resection and 35.2% for patients who underwent a subtotal resection.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a report from the Massachusetts General Hospital, 150 patients (aged <22 years) with WHO grade 2 and grade 3 ependymomas were treated with proton radiation therapy between 2001 and 2019. The median follow-up was 6.5 years.[18]

With multivariate analysis, the effect of tumor excision persisted after controlling for tumor location.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23]

In a combined Massachusetts General Hospital and University of Florida study, 386 children with nonmetastatic intracranial grade 2 and grade 3 ependymomas were treated with proton radiation therapy.[19]

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a combined Massachusetts General Hospital and University of Florida study, 386 children with nonmetastatic intracranial grade 2 and grade 3 ependymomas were treated with proton radiation therapy.[19]

With a median follow-up of 5 years, the 7-year local control rate was 77%, the PFS rate was 63.8%, and the OS rate was 82%.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a combined Massachusetts General Hospital and University of Florida study, 386 children with nonmetastatic intracranial grade 2 and grade 3 ependymomas were treated with proton radiation therapy.[19]

As with the previous report, subtotal resection was associated with worse local control, PFS, and OS.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a combined Massachusetts General Hospital and University of Florida study, 386 children with nonmetastatic intracranial grade 2 and grade 3 ependymomas were treated with proton radiation therapy.[19]

Radiation therapy doses of greater than 54 Gy were not associated with improved disease control or survival.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In a combined Massachusetts General Hospital and University of Florida study, 386 children with nonmetastatic intracranial grade 2 and grade 3 ependymomas were treated with proton radiation therapy.[19]

The rate of brain stem toxicity greater than grade 2 was 4%, and two children died of brain stem toxicity.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23]

In the KiProReg study, 105 children with intracranial ependymomas were treated with a median total dose of 59.4 Gy of proton radiation therapy. Children younger than 4 years received 54 Gy. The median follow-up was 1.9 years.[20]

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In the KiProReg study, 105 children with intracranial ependymomas were treated with a median total dose of 59.4 Gy of proton radiation therapy. Children younger than 4 years received 54 Gy. The median follow-up was 1.9 years.[20]

The estimated 3-year OS rate was 93.7%, the local control rate was 74.1%, and the PFS rate was 55.6%.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In the KiProReg study, 105 children with intracranial ependymomas were treated with a median total dose of 59.4 Gy of proton radiation therapy. Children younger than 4 years received 54 Gy. The median follow-up was 1.9 years.[20]

Multiple surgeries were identified as a risk factor for lower PFS.

Proton-beam radiation therapy (a type of charged-particle radiation therapy) provides a possible advantage for targeting the tumor (supratentorial or infratentorial) while avoiding critical normal brain and neuroendocrine tissues.Concerns about brain stem toxicity in very young children (aged <3 years) after proton therapy to the posterior fossa have prompted the use of more conservative doses in these children at some centers.[21-23] › In the KiProReg study, 105 children with intracranial ependymomas were treated with a median total dose of 59.4 Gy of proton radiation therapy. Children younger than 4 years received 54 Gy. The median follow-up was 1.9 years.[20]

There was a low rate of grade 3 toxicities and there were no episodes of symptomatic brain stem necrosis.

The International Society of Paediatric Oncology (SIOP) Ependymoma I study included 74 eligible pediatric patients with localized ependymomas. Thirty-three patients underwent a gross-total resection before receiving focal irradiation.[24][Level of evidence B4]

The International Society of Paediatric Oncology (SIOP) Ependymoma I study included 74 eligible pediatric patients with localized ependymomas. Thirty-three patients underwent a gross-total resection before receiving focal irradiation.[24][Level of evidence B4]

The 5-year EFS rate was 69%, and the 10-year EFS rate was 63%.

The International Society of Paediatric Oncology (SIOP) Ependymoma I study included 74 eligible pediatric patients with localized ependymomas. Thirty-three patients underwent a gross-total resection before receiving focal irradiation.[24][Level of evidence B4]

The 5-year OS rate was 81%, and the 10-year OS rate was 68%.

The International Society of Paediatric Oncology (SIOP) Ependymoma I study included 74 eligible pediatric patients with localized ependymomas. Thirty-three patients underwent a gross-total resection before receiving focal irradiation.[24][Level of evidence B4]

Post hoc analysis of known risk factors confirmed the impact of 1q gain, H3K27me3 loss, and hTERT expression.