Treatment and survival of supratentorial and posterior fossa ependymomas in adults

Nuño, Miriam A; Yu, Jeffrey; Varshneya, Kunal; Alexander, Julia; Mukherjee, Debraj; Black, Keith L.; Patil, Chirag G.

doi:10.1016/j.jocn.2015.11.014

Cited by 21 publications

(14 citation statements)

References 50 publications

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“…Cohorts of pediatric or adult patients in which the role of chemotherapy was retrospectively analyzed either failed to demonstrate a survival advantage or showed substantial variation between individual patients [3, 13, 28]. Two international randomized trials in children are currently comparing post-irradiation chemotherapy to observation only, SIOP Ependymoma II (Europe) and ACNS0831 (USA).…”

Section: Clinical Management Of Intracranial Ependymoma In the Contexmentioning

confidence: 99%

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

et al. 2016

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Multiple independent genomic profiling efforts have recently identified clinically and molecularly distinct subgroups of ependymoma arising from all three anatomic compartments of the central nervous system (supratentorial brain, posterior fossa, and spinal cord). These advances motivated a consensus meeting to discuss: (1) the utility of current histologic grading criteria, (2) the integration of molecular-based stratification schemes in future clinical trials for patients with ependymoma and (3) current therapy in the context of molecular subgroups. Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients with intracranial ependymoma (WHO Grade II/III) based on the knowledge of its molecular subgroups. The major consensus among attendees was reached that treatment decisions for ependymoma (outside of clinical trials) should not be based on grading (II vs III). Supratentorial and posterior fossa ependymomas are distinct diseases, although the impact on therapy is still evolving. Molecular subgrouping should be part of all clinical trials henceforth.

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Section: Clinical Management Of Intracranial Ependymoma In the Contexmentioning

confidence: 99%

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

et al. 2016

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“…59,62 A large hospital-based registry series, however, did not find a benefit to RT in adults. 63 More recently, 2 distinct molecular subgroups of posterior fossa ependymomas (excluding subependymomas) have been characterized, EPN_PFA and EPN_PFB. 34,37 EPN_PFA tumors have a poor prognosis and occur commonly in infants and young children, and EPN_PFB have a better prognosis and occur more often in older children and adults.…”

Section: Intracranial Ependymomasmentioning

confidence: 99%

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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“…54,55,57 Specific factors associated with worse survival in large retrospective series include higher histological grade, incomplete resection, ST location, and Karnofsky performance scale 80. 2,28,54,55,57 As expected with their WHO grade I histology, myxopapillary ependymomas have a better prognosis with an estimated 10-year PFS of 61.2% and 10-year survival of 92%. 58 Most of these patients are treated with surgery alone if a gross total resection has been achieved.…”

Section: Survivalmentioning

confidence: 65%

“…Retrospective analyses of pediatric and adult cohorts, in which chemotherapy was part of the treatment of ependymoma patients, either failed to demonstrate survival advantages linked to chemotherapy or showed substantial variation between individual patients. [26][27][28] In children, the two international randomized trials described in the following, Children's Oncology Group (COG) ACNS0831 and International Society for Pediatric Oncology (SIOP) Ependymoma II, are currently comparing postirradiation chemotherapy to observation only. Intracranial ependymomas mainly present with locally invasive growth patterns but low metastatic potential (<10% at the time of first diagnosis).…”

Section: Treatment Options For Intracranial Ependymomasmentioning

confidence: 99%

Ependymoma

Pajtler

Gerstner

2018

Semin Neurol

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Ependymoma can arise throughout the whole neuraxis. In children, tumors predominantly occur intracranially, whereas the spine is the most prevalent location in adults. Significant variance in the grade II versus grade III distinction of ependymomas has led to the acknowledgment that the clinical utility of histopathological classification is limited. Epigenomic profiling efforts have identified molecularly distinct groups of ependymomas that adequately reflect the biological, clinical, and histopathological heterogeneities across anatomical compartments, age groups, and grades. The recent update of the World Health Organization classification of central nervous system tumors has already integrated one of these groups, and molecular classification will be part of future clinical trials to improve risk stratification. Clinical management of this rare disease is challenging, making professional experience and intensified multidisciplinary cooperation pivotal factors for treatment success. Novel research strategies are currently applied for target discovery in ependymomas since for most molecular groups, genetic drivers are unknown.

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Treatment and survival of supratentorial and posterior fossa ependymomas in adults

Cited by 21 publications

References 50 publications

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

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

Case-based review: ependymomas in adults

Ependymoma

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