Adult Supratentorial Ependymoma: The Mayo Clinic Experience

Abstract

Purpose/Objective(s)To examine the role of radiotherapy (RT) on survival and recurrence in adult patients diagnosed with supratentorial ependymoma.Materials/MethodsA retrospective search of a Mayo Clinic tumor registry was conducted to identify patients diagnosed with primary intracranial ependymoma between 1969 and 2008. The extent of surgical resection was determined by intraoperative impression and post-operative imaging. The external beam radiotherapy (EBRT) dose used ranged from 50 to 72 Gy delivered at 1.75-3.12 Gy per fraction. The EBRT treatment volumes consisted of tumor bed only, whole brain only, whole brain with boost, and the entire CNS with or without boost to the tumor bed. Estimates of overall survival (OS) and recurrence-free survival (RFS) were determined from the date of initial surgery using the Kaplan-Meier method.ResultsA total of 42 adult patients (>18 y) were identified with a median age of 36.8 years (range 18.7-84.6). The median follow-up was 3.2 years (range 1.2 months to 13.9 years). 26 patients had Grade 2 and 14 had Grade 3 histology. At the time of analysis, 26 patients (62%) were alive. Kaplan-Meier estimates of OS and RFS at 5 years were 66% and 43%, respectively. The median survival was 9.7 years (range 1.2 months to 13.9 years). All patients (10) who had a subtotal resection (STR) received RT. 18 of 31 patients (58%) who underwent gross total resection (GTR) received RT. Twenty patients (48%) experienced a recurrence at a median of 3.2 years (range 1.2 months to 12.8 years). Three of 13 patients recurred after GTR without RT (range 0.9-4.6 yrs). Univariate analysis was performed to examine the role of presenting symptoms, grade, extent of resection, location, tumor size, radiation treatment, and chemotherapy treatment on OS and RFS. Significant factors predicting for improved OS included the extent of resection (p = 0.009), lack of recurrence (p = 0.02) and age < 40 years (p = 0.05). There were no significant factors in our cohort associated with RFS, however there was a trend towards decreased recurrence in patients who had hemispheric location of their tumor (p = 0.08) and in patients who had undergone a GTR of their initial tumor (p = 0.15).ConclusionsDespite our small numbers, this is the largest cohort of patients with supratentorial ependymoma in the literature. OS was improved in younger patients with GTR and who were without recurrence. GTR was not associated with improvement in RFS, likely because all patients with STR received post-operative RT. Previously reported prognostic factors (i.e. age, grade) were not associated with risk of recurrence suggesting that a better understanding of the molecular characteristics of supratentorial ependymomas is necessary to determine patients who require adjuvant radiotherapy. Purpose/Objective(s)To examine the role of radiotherapy (RT) on survival and recurrence in adult patients diagnosed with supratentorial ependymoma. To examine the role of radiotherapy (RT) on survival and recurrence in adult patients diagnosed with supratentorial ependymoma. Materials/MethodsA retrospective search of a Mayo Clinic tumor registry was conducted to identify patients diagnosed with primary intracranial ependymoma between 1969 and 2008. The extent of surgical resection was determined by intraoperative impression and post-operative imaging. The external beam radiotherapy (EBRT) dose used ranged from 50 to 72 Gy delivered at 1.75-3.12 Gy per fraction. The EBRT treatment volumes consisted of tumor bed only, whole brain only, whole brain with boost, and the entire CNS with or without boost to the tumor bed. Estimates of overall survival (OS) and recurrence-free survival (RFS) were determined from the date of initial surgery using the Kaplan-Meier method. A retrospective search of a Mayo Clinic tumor registry was conducted to identify patients diagnosed with primary intracranial ependymoma between 1969 and 2008. The extent of surgical resection was determined by intraoperative impression and post-operative imaging. The external beam radiotherapy (EBRT) dose used ranged from 50 to 72 Gy delivered at 1.75-3.12 Gy per fraction. The EBRT treatment volumes consisted of tumor bed only, whole brain only, whole brain with boost, and the entire CNS with or without boost to the tumor bed. Estimates of overall survival (OS) and recurrence-free survival (RFS) were determined from the date of initial surgery using the Kaplan-Meier method. ResultsA total of 42 adult patients (>18 y) were identified with a median age of 36.8 years (range 18.7-84.6). The median follow-up was 3.2 years (range 1.2 months to 13.9 years). 26 patients had Grade 2 and 14 had Grade 3 histology. At the time of analysis, 26 patients (62%) were alive. Kaplan-Meier estimates of OS and RFS at 5 years were 66% and 43%, respectively. The median survival was 9.7 years (range 1.2 months to 13.9 years). All patients (10) who had a subtotal resection (STR) received RT. 18 of 31 patients (58%) who underwent gross total resection (GTR) received RT. Twenty patients (48%) experienced a recurrence at a median of 3.2 years (range 1.2 months to 12.8 years). Three of 13 patients recurred after GTR without RT (range 0.9-4.6 yrs). Univariate analysis was performed to examine the role of presenting symptoms, grade, extent of resection, location, tumor size, radiation treatment, and chemotherapy treatment on OS and RFS. Significant factors predicting for improved OS included the extent of resection (p = 0.009), lack of recurrence (p = 0.02) and age < 40 years (p = 0.05). There were no significant factors in our cohort associated with RFS, however there was a trend towards decreased recurrence in patients who had hemispheric location of their tumor (p = 0.08) and in patients who had undergone a GTR of their initial tumor (p = 0.15). A total of 42 adult patients (>18 y) were identified with a median age of 36.8 years (range 18.7-84.6). The median follow-up was 3.2 years (range 1.2 months to 13.9 years). 26 patients had Grade 2 and 14 had Grade 3 histology. At the time of analysis, 26 patients (62%) were alive. Kaplan-Meier estimates of OS and RFS at 5 years were 66% and 43%, respectively. The median survival was 9.7 years (range 1.2 months to 13.9 years). All patients (10) who had a subtotal resection (STR) received RT. 18 of 31 patients (58%) who underwent gross total resection (GTR) received RT. Twenty patients (48%) experienced a recurrence at a median of 3.2 years (range 1.2 months to 12.8 years). Three of 13 patients recurred after GTR without RT (range 0.9-4.6 yrs). Univariate analysis was performed to examine the role of presenting symptoms, grade, extent of resection, location, tumor size, radiation treatment, and chemotherapy treatment on OS and RFS. Significant factors predicting for improved OS included the extent of resection (p = 0.009), lack of recurrence (p = 0.02) and age < 40 years (p = 0.05). There were no significant factors in our cohort associated with RFS, however there was a trend towards decreased recurrence in patients who had hemispheric location of their tumor (p = 0.08) and in patients who had undergone a GTR of their initial tumor (p = 0.15). ConclusionsDespite our small numbers, this is the largest cohort of patients with supratentorial ependymoma in the literature. OS was improved in younger patients with GTR and who were without recurrence. GTR was not associated with improvement in RFS, likely because all patients with STR received post-operative RT. Previously reported prognostic factors (i.e. age, grade) were not associated with risk of recurrence suggesting that a better understanding of the molecular characteristics of supratentorial ependymomas is necessary to determine patients who require adjuvant radiotherapy. Despite our small numbers, this is the largest cohort of patients with supratentorial ependymoma in the literature. OS was improved in younger patients with GTR and who were without recurrence. GTR was not associated with improvement in RFS, likely because all patients with STR received post-operative RT. Previously reported prognostic factors (i.e. age, grade) were not associated with risk of recurrence suggesting that a better understanding of the molecular characteristics of supratentorial ependymomas is necessary to determine patients who require adjuvant radiotherapy.