The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants

Pajtler, Kristian W.; Mack, Stephen C.; Ramaswamy, Vijay; Smith, Christian A.; Witt, Hendrik; Smith, Amy; Hansford, Jordan R.; Hoff, Katja von; Wright, Karen; Hwang, Eui-Hyoung; Frappaz, Didier; Kanemura, Yonehiro; Massimino, Maura; Faure‐Conter, Cécile; Modena, Piergiorgio; Tabori, Uri; Warren, Katherine E.; Holland, Eric C.; Ichimura, Koichi; Giangaspero, Felice; Castel, David; Deimling, Andreas von; Kool, Marcel; Dirks, Peter B.; Grundy, Richard G.; Foreman, Nicholas K.; Gajjar, Amar; Korshunov, Andrey; Finlay, Jonathan L.; Gilbertson, Richard J.; Ellison, David W.; Aldape, Kenneth; Merchant, Thomas E.; Bouffet, Éric; Pfister, Stefan M.; Taylor, Michael D.

doi:10.1007/s00401-016-1643-0

Cited by 286 publications

(266 citation statements)

References 40 publications

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“…Therefore, as different diseases, treatment decisions for patients with ependymomas outside of clinical trials should not be based on WHO grading (II vs III). 44 …”

Section: Pathologymentioning

confidence: 99%

“…Current consensus guidelines recommend against using tumor grading in treatment decisions because of poor reproducibility and incorporating molecular subgrouping in clinical trials, although this remains controversial. 44,67 Thus, RT should be given for patients with residual tumor after surgery, while clinical trials should evaluate the benefit of RT in the setting of GTR.…”

Section: Intracranial Ependymomasmentioning

confidence: 99%

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Case-based review: ependymomas in adults

Cachia

Johnson

Kaufmann

et al. 2018

Neuro-Oncology Practice

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Ependymomas are rare primary central nervous system (CNS) tumors in adults. They occur most commonly in the spinal cord, and have classically been graded histologically into World Health Organization (WHO) grades I, II, or III based on the level of anaplasia. Recent data are showing that genetic heterogeneity occurs within the same histological subgroup and that ependymomas arising from different CNS locations have different molecular signatures. This has renewed interest in developing targeting therapies based on molecular profiles especially given the variable outcomes with radiation and the poor results with cytotoxic agents. In this paper, we present the case of a 46-year-old woman with a classic presentation of spinal cord ependymoma and discuss the current histopathological and molecular classification for ependymomas as well as current guidelines for patient management.

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“…Therefore, as different diseases, treatment decisions for patients with ependymomas outside of clinical trials should not be based on WHO grading (II vs III). 44 …”

Section: Pathologymentioning

confidence: 99%

Section: Intracranial Ependymomasmentioning

confidence: 99%

Case-based review: ependymomas in adults

Cachia

Johnson

Kaufmann

et al. 2018

Neuro-Oncology Practice

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“…Examples may include meningioma, atypical teratoid/rhabdoid tumor, diffuse astrocytoma IDH wild type, and pilocytic astrocytoma. Other molecular tumor types have not yet been introduced into the WHO Classification system, although they have been already included in consensus suggestions on clinical management, such as ependymoma with YAP fusion or infratentorial ependymoma types A and B [7]. Furthermore, new molecular or surrogate markers that are important for classification and diagnosis will be developed.…”

Section: Economics or Ethics?mentioning

confidence: 99%

WHO 2016: Open questions and practical implications

Paulus

2017

Acta Neurochir

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The WHO Classification of Tumors of the Central Nervous System has been a story of success over the past decades. There is general agreement that the WHO Classification represents the basis not only for neuropathological diagnosis but also for prognosis and therapy. The world-wide consensus of using this classification system is a remarkable achievement and may serve as a role model for communities working on other neurological diseases where heterogeneous and conflicting classification systems impair scientific and clinical progress.The most recent WHO Classification has been issued in May 2016 and includes several changes, as detailed in the BBlue Book^ [3] and summarized in an accompanying review article [4] as well as in the paper by Banan and Hartmann published in this issue of Acta Neurochirurgica. As in previous updates of the WHO Classification, a few newly recognized clinico-pathological tumor types have been added, such as diffuse leptomeningeal glioneuronal tumor, epithelioid glioblastoma, and anaplastic pleomorphic xanthoastrocytoma. A few others were deleted, such as protoplasmic astrocytoma, cellular ependymoma, and gliomatosis cerebri. Other tumors were renamed, including fibrillary astrocytoma as diffuse astrocytoma, hemangiopericytoma as solitary fibrous tumor/ hemangiopericytoma, and primitive neuroectodermal tumor (PNET) as CNS embryonal tumor. Tumors that have been known (and diagnosed) for a long time before but somehow had been Bforgotten^in previous WHO Classifications have been added in chapters on lymphomas, histiocytic tumors, tumors of the cranial and paraspinal nerves, and mesenchymal tumors. Finally, brain invasion has been clearly defined as being sufficient for making the diagnosis of atypical meningioma.However, the most dramatic change, which is unparalleled by previous editions, is the inclusion of molecular data in the diagnosis of several tumor types (actually still being a minority), resulting in integrated histological/molecular diagnoses. Examples include Boligodendroglioma, IDH-mutant and 1p/ 19q-codeleted, grade II^and Bclassical medulloblastoma, SHH-activated and TP53 mutant, grade IV^. Compared to the conventional, relatively broad tumor types solely defined by histology, the narrowly defined, combined histological/ molecular newcomers will lead to more homogeneous tumor groups, which are expected to be advantageous for clinical studies and eventually for the management of individual patients. While the updated classification is straightforward, clinically relevant, and based on recent molecular insight, it provokes questions concerning practical application. A few of these questions are listed below, not necessarily complemented with an unequivocal answer.Is 1p/19q analysis required for all cases of diffuse glioma?Codeletion of the short arm of chromosome 1 and the long arm of chromosome 19 is currently considered a defining molecular feature of oligodendroglioma. While in cases of histologically classical oligodendroglioma 1p/19q analysis is essential for making the f...

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“…subependymomas and myxopapillary ependymoma), histopathologic grading has no prognostic utility. Therefore we recommend that outside of current clinical trials, histopathologic grading of Grade II or III ependymoma should not be used to risk stratify future patients[10] (Figure 1). …”

Section: Introductionmentioning

confidence: 99%

Put away your microscopes: the ependymoma molecular era has begun

Mack

Taylor

2017

Current Opinion in Oncology

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Purpose of review To synthesize, integrate, and comment on recent research developments to our understanding of the molecular basis of ependymoma, and to place this in context with current treatment and research efforts. Recent findings Our recent understanding of the histologically defined molecular entity ependymoma has rapidly advanced through genomic, transcriptomic, and epigenomic profiling studies. Summary These advancements lay the groundwork for development of future ependymoma biomarkers, models, and therapeutics. Our review discusses these discoveries and their impact on our clinical understanding of this disease. Lastly, we offer insight into clinical and research areas requiring further validation, and open questions remaining in the field.

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The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants

Cited by 286 publications

References 40 publications

Case-based review: ependymomas in adults

Case-based review: ependymomas in adults

WHO 2016: Open questions and practical implications

Put away your microscopes: the ependymoma molecular era has begun

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