Ependymoma

Dorfer, Christian; Tonn, Joerg C.; Rutka, James T.

doi:10.1016/b978-0-12-802997-8.00025-6

Cited by 20 publications

(6 citation statements)

References 94 publications

(90 reference statements)

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“…15 While the cell of origin in the dog is not known, studies of human ependymoma reveal features of radial glia, a neural stem cell that resides in the subventricular zone. 4 Ependymomas are typically well-circumscribed intraventricular neoplasms that do not extend extensively into the adjacent parenchyma. Pseudorosettes and rosettes are an important histologic feature of these tumors; however, they are not specific to ependymomas, and using them as the sole criterion for Abbreviations: FS, female spayed; M, male, not otherwise specified; MC, male castrated; MI, male intact; NA, not applicable.…”

Section: Discussionmentioning

confidence: 99%

“…This is consistent with the staining pattern of Olig2 in human ependymomas, in which a minor subset of neoplastic cells has expression of Olig2. 4,5 Since this pattern of Olig2 expression is expected with radial glia, it is often used to support the contention that these tumors arise from a subpopulation of radial glia stem cells. 4,5 S100 immunolabeling was present in 3 of 4 of the cases, and while certainly not specific to ependymoma, the multifocal, intense cytoplasmic staining pattern was consistent with that reported in human ependymoma.…”

Section: Discussionmentioning

confidence: 99%

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Canine Ependymoma: Diagnostic Criteria and Common Pitfalls

et al. 2019

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Reports of canine ependymoma are generally restricted to single case reports with tumor incidence estimated at 2% to 3% of primary central nervous system (CNS) tumors. While most commonly reported in the lateral ventricle, tumors can occur anywhere in the ventricular system and in extraventricular locations. Rosettes and pseudorosettes are a common histologic feature; however, these features can be mimicked by other CNS neoplasms. Thirty-seven potential ependymoma cases were identified in a retrospective database search of 8 institutions, and a histologic review of all cases was conducted. Of 37 cases, 22 candidate cases were further subjected to a consensus histologic and immunohistochemical review, and only 5 of 37 (13.5%) were conclusively identified as ependymoma. The neuroanatomic locations were the lateral ventricle (3/5), third ventricle (1/5), and mesencephalic aqueduct (1/5). Subtypes were papillary (4/5) and tanycytic (1/5). Histologic features included rosettes (5/5), pseudorosettes (5/5), ependymal canals (2/5), tanycytic differentiation (1/5), blepharoplasts (1/5), ciliated cells (1/5), and high nuclear to cytoplasmic ratio (5/5). Immunolabeling for GFAP (4/4) and CKAE1/3 (3/4) was found in pseudorosettes, rosettes, and scattered individual neoplastic cells. Diffuse but variably intense cytoplasmic S100 immunolabeling was detected in 3 of 4 cases. Olig2 intranuclear immunolabeling was observed in less than 1% of the neoplastic cells (3/3). Tumors that had pseudorosettes and mimicked ependymoma included oligodendroglioma, choroid plexus tumor, pituitary corticotroph adenoma, papillary meningioma, and suprasellar germ cell tumor. These findings indicate that canine ependymoma is an extremely rare neoplasm with histomorphologic features that overlap with other primary CNS neoplasms.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Canine Ependymoma: Diagnostic Criteria and Common Pitfalls

et al. 2019

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“…E pendymomas (EPNs) are rare glial cell tumors that involve 3 major anatomic compartments (supratentorial [ST], posterior fossa [PF], and spinal cord) of the central nervous system (CNS) and affect both children and adults. 1 The current treatment strategy for patients with ependymal tumor remains maximal safe surgical resection, followed by focal radiotherapy. Despite histopathologic features are similar, the clinical behavior and outcome of ependymal tumors are highly variable.…”

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confidence: 99%

Survival and Prognostic Factors of Adult Intracranial Ependymoma

Zhao

Wu²,

Wang

et al. 2021

American Journal of Surgical Pathology

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Adult intracranial ependymomas (EPNs) are extremely rare brain tumors. Currently, clinical and molecular factors that could inform individualized treatment strategies are still lacking for EPNs in this age group. The aim of this study was to investigate potential prognostic indicators and rational therapeutic management in a large cohort of adult intracranial EPNs. Adult patients who underwent resection of World Health Organization (WHO) grade II or III intracranial EPNs were included. The demographic features, clinicopathologic manifestations, molecular subgroups, and outcomes were retrospectively analyzed. Overall survival and progression-free survival were calculated using the Kaplan-Meier analysis. Potential prognostic indicators were identified using multivariable Cox proportional hazards model. This cohort included 236 adult patients with a mean age of 36.2 years (range: 18 to 72 y) at diagnosis. The tumor location was supratentorial (ST) in 102 (43.2%) and infratentorial in 134 (56.8%). Pathologic analysis revealed 43.1% of ST-EPNs with RELA fusion and 88.1% of posterior fossa ependymomas (PF-EPNs) with positive H3K27me3 staining. Gross total removal was achieved in 169 cases (71.6%). During follow-up, 97 (41.1%) patients had disease progression and 39 (16.5%) died. Kaplan-Meier analysis showed that patients with H3K27me3-positive PF-EPN had excellent survival, whereas patients with RELA fusion-positive ST-EPN or H3K27me3-negative PF-EPN had poor prognosis (progression-free survival: P=1.3E−16, overall survival: P=2.5E−12). Multivariate analysis showed that molecular subgroup, extent of resection, and Ki-67 index were strong independent prognostic indicators. In conclusion, our study provides essential information on the prognostic prediction of adult intracranial EPNs that will assist in establishing appropriate risk stratification and individualized treatment strategies in future clinical trials.

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“…Ependymomas are a heterogeneous group of primary central nervous system (CNS) tumors with multiple histological, brain region, age, and molecular features distinguishing between different prognostic groups ( Pajtler et al 2015 ; Dorfer et al 2016 ; Khatua et al 2017 ). Based on standard histological features, ependymal neoplasms can be diagnosed as World Health Organization (WHO) Grade I, II, or III tumors.…”

Section: Introductionmentioning

confidence: 99%

Resistance-promoting effects of ependymoma treatment revealed through genomic analysis of multiple recurrences in a single patient

Miller

Dahiya

et al. 2018

Cold Spring Harb Mol Case Stud

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As in other brain tumors, multiple recurrences after complete resection and irradiation of supratentorial ependymoma are common and frequently result in patient death. This standard-of-care treatment was established in the pregenomic era without the ability to evaluate the effect that mutagenic therapies may exert on tumor evolution and in promoting resistance, recurrence, and death. We seized a rare opportunity to characterize treatment effects and the evolution of a single patient's ependymoma across four recurrences after different therapies. A combination of high-depth whole-genome and exome-based DNA sequencing of germline and tumor specimens, RNA sequencing of tumor specimens, and advanced computational analyses were used. Treatment with radiation and chemotherapies resulted in a substantial increase in mutational burden and diversification of the tumor subclonal architecture without eradication of the founding clone. Notable somatic alterations included a MEN1 driver, several epigenetic modifiers, and therapy-induced mutations that impacted multiple other cancer-relevant pathways and altered the neoantigen landscape. These genomic data provided new mechanistic insights into the genesis of ependymoma and pathways of resistance. They also revealed that radiation and chemotherapy were significant forces in shaping the increased subclonal complexity of each tumor recurrence while also failing to eradicate the founding clone. This raises the question of whether standard-of-care treatments have similar consequences in other patients with ependymoma and other types of brain tumors. If so, the perspective obtained by real-time genomic characterization of a tumor may be essential for making effective patient-specific and adaptive clinical decisions.

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Ependymoma

Cited by 20 publications

References 94 publications

Canine Ependymoma: Diagnostic Criteria and Common Pitfalls

Canine Ependymoma: Diagnostic Criteria and Common Pitfalls

Survival and Prognostic Factors of Adult Intracranial Ependymoma

Resistance-promoting effects of ependymoma treatment revealed through genomic analysis of multiple recurrences in a single patient

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