Abstract:BACKGROUND:
There is limited data from Saudi Arabia on the demographic characteristics, outcomes and effectiveness of different treatment modalities in children with intracranial ependymoma.
OBJECTIVE:
Study the characteristics of pediatric ependymoma and outcomes of treatment modalities in Saudi Arabia.
DESIGN:
Retrospective.
SETTING:
Tertiary care center.
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“…The management of children with ependymoma is still a controversial topic in paediatric neuro-oncology and may vary among institutions, from only-surgery to a combination of surgery, RTH, CTH or even autologous hematopoietic stem cell transplant (autoHSCT, Table A1 ) [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , …”
Section: Discussionmentioning
confidence: 99%
“…et al (2021) [ 54 ]/R 386 Cranial S + pRTH + CTH (30%) 7 y PFS, OS: 64%, 82% R0, female sex, infratentorial site Lundar T. et al (2021) [ 55 ]/R 26 Cranial S + RTH (69%) + CTH (11%) 5 y OS: 26% (early period of the study), 73% (late period) Later time of the treatment, MRI-era Lundar T. et al (2021) [ 56 ]/R 33 Spinal S + RTH (27%) 10 y OS: 67% Not found Shah S.N. et al (2020) [ 57 ]/R 22 Cranial S + RTH (77%) + CTH (23%) 5 y OS: 55% Not found Massimino M. et al (2021) [ 58 ]/P * 160 Cranial S + RTH + CTH 5, 10 y PFS & OS: 66%, 58% & 80%, 73% R0, lower histologic G, molecular features Abbreviations: &—and; AHSCT—autologous hematopoietic cell transplant; CSS—cancer specific survival; CTH—chemotherapy; FFS—failure-free survival; G—grade; hRTH—hypofractionated radiotherapy; IMRT—intensity modulated radiotherapy; mets—metastases; MRI—magnetic resonance imaging; N—number of patients; OS—overall survival; P—prospective; PFS—progression-free survival; pRTH—proton radiotherapy; R—retrospective; RTH—radiotherapy; R0—radical resection (macro- and microscopically); R1—macroscopically radical resection (but not microscopically); R2—macroscopically not-radical resection; S—surgery; SRS—stereotactic radiosurgery; y—year; y/o—year old; vs.—versus; * update of the study from 2016, reference number 10. Figure A1 The schema of the study—treatment applied and outcome of th...…”
We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/−CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.
“…The management of children with ependymoma is still a controversial topic in paediatric neuro-oncology and may vary among institutions, from only-surgery to a combination of surgery, RTH, CTH or even autologous hematopoietic stem cell transplant (autoHSCT, Table A1 ) [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , …”
Section: Discussionmentioning
confidence: 99%
“…et al (2021) [ 54 ]/R 386 Cranial S + pRTH + CTH (30%) 7 y PFS, OS: 64%, 82% R0, female sex, infratentorial site Lundar T. et al (2021) [ 55 ]/R 26 Cranial S + RTH (69%) + CTH (11%) 5 y OS: 26% (early period of the study), 73% (late period) Later time of the treatment, MRI-era Lundar T. et al (2021) [ 56 ]/R 33 Spinal S + RTH (27%) 10 y OS: 67% Not found Shah S.N. et al (2020) [ 57 ]/R 22 Cranial S + RTH (77%) + CTH (23%) 5 y OS: 55% Not found Massimino M. et al (2021) [ 58 ]/P * 160 Cranial S + RTH + CTH 5, 10 y PFS & OS: 66%, 58% & 80%, 73% R0, lower histologic G, molecular features Abbreviations: &—and; AHSCT—autologous hematopoietic cell transplant; CSS—cancer specific survival; CTH—chemotherapy; FFS—failure-free survival; G—grade; hRTH—hypofractionated radiotherapy; IMRT—intensity modulated radiotherapy; mets—metastases; MRI—magnetic resonance imaging; N—number of patients; OS—overall survival; P—prospective; PFS—progression-free survival; pRTH—proton radiotherapy; R—retrospective; RTH—radiotherapy; R0—radical resection (macro- and microscopically); R1—macroscopically radical resection (but not microscopically); R2—macroscopically not-radical resection; S—surgery; SRS—stereotactic radiosurgery; y—year; y/o—year old; vs.—versus; * update of the study from 2016, reference number 10. Figure A1 The schema of the study—treatment applied and outcome of th...…”
We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/−CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.
IntroductionEpendymoma is the third most frequent central malignant nervous system tumor in the pediatric age group. There is scarce data in the literature on survival of these patients, especially in upper and lower middle-income countries. We aimed to describe the clinical and demographic characteristics, treatment, and outcome of pediatric patients with ependymoma admitted to a public cancer hospital.MethodsRetrospective analysis of medical records of patients with ependymoma, admitted to the Pediatric Oncology department (0-20 years) during the period of 2000-2022. Data on patient, disease characteristics, and treatment were analyzed. Overall survival (OS) was calculated using the Kaplan-Meier method.ResultsSeventy-two patients were evaluated; median age at diagnosis was 6.5 years (range: 1-20), 63% were male, 54% of the tumors were in the posterior fossa (PF-EPN), 45% were classified as WHO grade 3, and 68% were operated on in other institutions before referral. Regarding treatment, 72% underwent radiotherapy and 33% of patients underwent chemotherapy. Almost 70% percent of the patients had relapses. The median follow-up time was 5.2 years (Range: 0,1-21,4). The OS in 5 years was 67%. Totally resected tumors had OS in 5 years of 88% (p: 0.028).ConclusionThe results achieved in this series show a survival gap between UMIC and HIC. Relapses occurred mainly in the first ten years and then reached a plateau, with the majority of patients experiencing endocrinological and neurological sequelae.
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