Pediatric intracranial ependymoma: the roles of surgery, radiation and chemotherapy

Abstract

Management of pediatric intracranial ependymomas poses a major challenge, and optimal treatment remains controversial. We sought to investigate the roles of surgery, radiation, and chemotherapy in a historical cohort. Thirty-nine children, age 21 or younger, with non-metastatic intracranial ependymomas were treated from 1972 to 2008. Median age was 8 years (range 0.2-19.1). Twenty-one patients (54%) underwent GTRs, and 18 (45%) underwent STRs. Twenty-six patients (67%) received upfront adjuvant RT (67%), and 14 (44%) received adjuvant chemotherapy. Twenty-four patients had disease recurrence and 12 died. Only one patient recurred after 5 years. Median PFS was 2.7 years and median OS was 20 years. Fifteen year PFS and OS were 30 and 67%. Adjuvant RT was associated with improved PFS (P = 0.045), and remained significant after adjusting for EOR (P = 0.04). Greater EOR trended towards prolonged survival, but did not reach statistical significance (P = 0.156). Of the patients that underwent GTR, the median PFS was 38 months for those treated with adjuvant RT versus 30 months for those that were not treated with RT. Of the patients that had STR, the median PFS for those treated with RT was 26.3 months versus 6.9 months for those were not treated with RT. In conclusion, for localized intracranial pediatric ependymomas, adjuvant RT is associated with improved PFS, even after adjusting for EOR. Our findings suggest the benefit of RT even in the presence of GTR. Future prospective studies with larger sample number are needed to validate our findings.

Progression‐free survival of children with localized ependymoma treated with intensity‐modulated radiation therapy or proton‐beam radiation therapy

“…[1][2][3][4] In most series, the degree of surgical resection is consistently the most important prognostic factor for local control and survival, and current 3-to 5-year survival rates range from 60% to 85%. The standard treatment for ependymoma is maximal surgical resection followed by involved-field radiation therapy (RT).…”

Chromosome 1q gain and tenascin-C expression are candidate markers to define different risk groups in pediatric posterior fossa ependymoma

“…In contrast to adults where spinal localization predominates, ependymomas in children are located predominantly intracranially, and most commonly in the posterior fossa [2]. Outcome of pediatric patients with intracranial ependymoma remains relatively poor with 5-year progression free survival rates between 30 % and 69.1 % and 5 year overall survival rates between 60 and 81 % in different age groups and therapeutic trial cohorts [4–7]. Among clinical factors the extent of resection is the most important prognostic factor for patient outcome [8], but age below 3 years and infratentorial location have been also suggested as unfavorable prognostic markers [9, 10].…”

Incus Necrosis After Irradiation

“…Survival is reported as 50% to 75% at 5 years (11,12). Malignant ependymomas may be treated with a combination of surgery, radiation therapy, and chemotherapy.…”