A brief history of ependymoma

Abstract

Ependymoma represents a biologically and clinically heterogeneous group of glial tumours that arise throughout the whole neuroaxis and in all age groups. Whereas intracranial ependymoma is usually found in children, adults suffer mostly from spinal cord ependymoma. In comparison to other neuro-oncological tumour entities, ependymoma has been largely understudied for decades. However, the recent years resulted in unprecedented progress with regard to the understanding of the biological underpinnings and clinical features of ependymoma. Here, we review the history of ependymoma research with a focus on the development of classification models throughout the years and a discussion of the most important clinical trials that have led to the current therapeutic standard, comprising maximal safe resection and, in most cases, radiotherapy. Critically, the evidence for effective drugs and chemotherapies in ependymoma is still sparse. However, these important questions may be soon addressed with the finalisation of the currently unpublished last generation of multi-institutional trials in Europe (SIOP EP II) and Northern America (ACNS0831). Lastly, we discuss the current challenges in the field of ependymoma research and the necessary next steps towards the goal of findings cures for all types of ependymal tumours.

Temporal trends and short term prediction of relative survival in ependymoma using model based period analysis

“…

BackgroundEpendymoma is a rare central nervous system tumor with substantial heterogeneity in anatomical site, histology, and clinical outcome [1][2][3] . Population-based evidence on temporal changes in survival remains limited, and up-to-date estimates are particularly needed 4,5 .

…”

“…Ependymoma is a rare tumor of the central nervous system that arises throughout the neuraxis and affects both children and adults 3,[12][13][14] . Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent.…”

“…Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent. Although gross total resection remains a cornerstone of management, prognosis differs substantially across patient subgroups, and long-term survival patterns at the population level remain incompletely characterized 3,4,12 .…”

Temporal trends and short term prediction of relative survival in ependymoma using model based period analysis

“…

BackgroundEpendymoma is a rare central nervous system tumor with substantial heterogeneity in anatomical site, histology, and clinical outcome [1][2][3] . Population-based evidence on temporal changes in survival remains limited, and up-to-date estimates are particularly needed 4,5 .

…”

“…Ependymoma is a rare tumor of the central nervous system that arises throughout the neuraxis and affects both children and adults 3,[12][13][14] . Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent.…”

“…Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent. Although gross total resection remains a cornerstone of management, prognosis differs substantially across patient subgroups, and long-term survival patterns at the population level remain incompletely characterized 3,4,12 .…”

Temporal trends and short term prediction of relative survival in ependymoma using model based period analysis

“…

BackgroundEpendymoma is a rare central nervous system tumor with substantial heterogeneity in anatomical site, histology, and clinical outcome [1][2][3] . Population-based evidence on temporal changes in survival remains limited, and up-to-date estimates are particularly needed 4,5 .

…”

“…Ependymoma is a rare tumor of the central nervous system that arises throughout the neuraxis and affects both children and adults 3,[12][13][14] . Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent.…”

“…Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent. Although gross total resection remains a cornerstone of management, prognosis differs substantially across patient subgroups, and long-term survival patterns at the population level remain incompletely characterized 3,4,12 .…”

Temporal trends and short term prediction of relative survival in ependymoma using model based period analysis

“…

BackgroundEpendymoma is a rare central nervous system tumor with substantial heterogeneity in anatomical site, histology, and clinical outcome [1][2][3] . Population-based evidence on temporal changes in survival remains limited, and up-to-date estimates are particularly needed 4,5 .

…”

“…Ependymoma is a rare tumor of the central nervous system that arises throughout the neuraxis and affects both children and adults 3,[12][13][14] . Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent.…”

“…Its clinical behavior is highly heterogeneous, re ecting variation in anatomical site, histological subtype, age at diagnosis, and disease extent. Although gross total resection remains a cornerstone of management, prognosis differs substantially across patient subgroups, and long-term survival patterns at the population level remain incompletely characterized 3,4,12 .…”