IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?

Objective: The effective treatment of posterior fossa ependymomas is surgery followed by radio-chemotherapy. Our aim is to evaluate the effects of sex, age, methylation subgrouping, extent of resection, radiation treatment (RT), MIB-1 index, grade, ATRX and H3K27M mutations on prognosis in pediatric...

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Autores principales:	Bahattin Tanrıkulu, Ayça Erşen Danyeli, Cengiz Canpolat, M. Memet Özek
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/04224694b33c48e1a801b380c7198dbf
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spelling	oai:doaj.org-article:04224694b33c48e1a801b380c7198dbf2021-11-10T04:34:08ZIS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?2531-137910.1016/j.htct.2021.10.1004https://doaj.org/article/04224694b33c48e1a801b380c7198dbf2021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2531137921011512https://doaj.org/toc/2531-1379Objective: The effective treatment of posterior fossa ependymomas is surgery followed by radio-chemotherapy. Our aim is to evaluate the effects of sex, age, methylation subgrouping, extent of resection, radiation treatment (RT), MIB-1 index, grade, ATRX and H3K27M mutations on prognosis in pediatric patients with posterior fossa ependymoma (PFE). Methodology: This is a retrospective study. Forty-two children with PFE who had surgery in our institution between 1996 and 2018 were included. Formalin-fixed paraffin-embedded tumor samples were evaluated for H3K27me3 immunostaining, MIB-1 index, WHO grades, ATRX and H3K27M mutations.Samples with global H3K27me3 reduction were grouped as posterior fossa ependymoma group A (PFA), whereas tumor samples with H3K27me3 nuclear immunopositivity were grouped as posterior fossa ependymoma group B (PFB). Results: Mean age of patients was 4.4 years (range 0.71-14.51). Thirty-one patients (73.8%) were PFA, whereas 11 patients (26.2%) were PFB. WHO grades of PFAs were statistically higher in comparison to WHO grades of PFBs. There are no significant differences between PFAs and PFBs in terms of resection rates, disease recurrence and survival parameters.Patients with total surgical excisions had significantly better PFS and OS rates. Conclusion: Extent of surgical excision is the most important prognostic indicator in PFEs. Prognostic effect of methylation subgrouping may be minimized with more aggressive surgical strategy in PFAs.Bahattin TanrıkuluAyça Erşen DanyeliCengiz CanpolatM. Memet ÖzekElsevierarticleDiseases of the blood and blood-forming organsRC633-647.5ENHematology, Transfusion and Cell Therapy, Vol 43, Iss , Pp S30- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Diseases of the blood and blood-forming organs RC633-647.5 Bahattin Tanrıkulu Ayça Erşen Danyeli Cengiz Canpolat M. Memet Özek IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
description	Objective: The effective treatment of posterior fossa ependymomas is surgery followed by radio-chemotherapy. Our aim is to evaluate the effects of sex, age, methylation subgrouping, extent of resection, radiation treatment (RT), MIB-1 index, grade, ATRX and H3K27M mutations on prognosis in pediatric patients with posterior fossa ependymoma (PFE). Methodology: This is a retrospective study. Forty-two children with PFE who had surgery in our institution between 1996 and 2018 were included. Formalin-fixed paraffin-embedded tumor samples were evaluated for H3K27me3 immunostaining, MIB-1 index, WHO grades, ATRX and H3K27M mutations.Samples with global H3K27me3 reduction were grouped as posterior fossa ependymoma group A (PFA), whereas tumor samples with H3K27me3 nuclear immunopositivity were grouped as posterior fossa ependymoma group B (PFB). Results: Mean age of patients was 4.4 years (range 0.71-14.51). Thirty-one patients (73.8%) were PFA, whereas 11 patients (26.2%) were PFB. WHO grades of PFAs were statistically higher in comparison to WHO grades of PFBs. There are no significant differences between PFAs and PFBs in terms of resection rates, disease recurrence and survival parameters.Patients with total surgical excisions had significantly better PFS and OS rates. Conclusion: Extent of surgical excision is the most important prognostic indicator in PFEs. Prognostic effect of methylation subgrouping may be minimized with more aggressive surgical strategy in PFAs.
format	article
author	Bahattin Tanrıkulu Ayça Erşen Danyeli Cengiz Canpolat M. Memet Özek
author_facet	Bahattin Tanrıkulu Ayça Erşen Danyeli Cengiz Canpolat M. Memet Özek
author_sort	Bahattin Tanrıkulu
title	IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
title_short	IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
title_full	IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
title_fullStr	IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
title_full_unstemmed	IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?
title_sort	is methylation status subgrouping really a strong prognostic factor in pediatric posterior fossa ependymoma?
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/04224694b33c48e1a801b380c7198dbf
work_keys_str_mv	AT bahattintanrıkulu ismethylationstatussubgroupingreallyastrongprognosticfactorinpediatricposteriorfossaependymoma AT aycaersendanyeli ismethylationstatussubgroupingreallyastrongprognosticfactorinpediatricposteriorfossaependymoma AT cengizcanpolat ismethylationstatussubgroupingreallyastrongprognosticfactorinpediatricposteriorfossaependymoma AT mmemetozek ismethylationstatussubgroupingreallyastrongprognosticfactorinpediatricposteriorfossaependymoma
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IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?

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