Cortically based cystic supratentorial RELA fusion-positive ependymoma: a case report with unusual presentation and appearance and review of literature

Sallam, Yasmine; Zhang, Qi; Pandey, Sachin

doi:10.1016/j.radcr.2020.09.022

Cited by 4 publications

(3 citation statements)

References 27 publications

(34 reference statements)

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“…ST-ZFTA is the second main subgroup of ependymoma in the ST compartment and harbors the worst outcome [ 1 ]. ST-ZFTA has a predilection for the lateral and third ventricle, with most occurring adjacent to the ventricular system, but it is not uncommon for this subset of ependymoma to be extraventricular and cortically based [ 178 ]. While the median age of patients with this tumor subgroup is 8 years, the range is 0 to 69 years, with 23% being adults and a peak incidence in the sixth decade of life for those diagnosed in adulthood [ 34 , 40 ].…”

Section: Supratentorial Ependymoma With Zfta-fusion (St-zfta)mentioning

confidence: 99%

Molecular Classification and Therapeutic Targets in Ependymoma

Larrew

Saway

Lowe³

et al. 2021

Cancers

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Ependymoma is a biologically diverse tumor wherein molecular classification has superseded traditional histological grading based on its superior ability to characterize behavior, prognosis, and possible targeted therapies. The current, updated molecular classification of ependymoma consists of ten distinct subgroups spread evenly among the spinal, infratentorial, and supratentorial compartments, each with its own distinct clinical and molecular characteristics. In this review, the history, histopathology, standard of care, prognosis, oncogenic drivers, and hypothesized molecular targets for all subgroups of ependymoma are explored. This review emphasizes that despite the varied behavior of the ependymoma subgroups, it remains clear that research must be performed to further elucidate molecular targets for these tumors. Although not all ependymoma subgroups are oncologically aggressive, development of targeted therapies is essential, particularly for cases where surgical resection is not an option without causing significant morbidity. The development of molecular therapies must rely on building upon our current understanding of ependymoma oncogenesis, as well as cultivating transfer of knowledge based on malignancies with similar genomic alterations.

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Section: Supratentorial Ependymoma With Zfta-fusion (St-zfta)mentioning

confidence: 99%

Molecular Classification and Therapeutic Targets in Ependymoma

Larrew

Saway

Lowe³

et al. 2021

Cancers

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“…In several literatures, CEs are defined as EPNs that are located in the cerebral cortex and have no connection to the ventricular lining and that account for only about 3.5% to 5.7% of all EPNs [ 2 3 14 15 ]. Especially, in some literatures like this case, it is called pure CEs to emphasize that they are limited only to the cerebral cortex [ 5 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…They are most frequently seen in children and young adults [ 1 2 ]. Only a third of them are supratentorial [ 3 ]. The most common subtype of supratentorial ependymoma (STE) is the C11orf95-RELA -fusion type [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Pure Cortical Ependymoma With RELA-Fusion Positive in a Young Adult

Lee

et al. 2021

Brain Tumor Res Treat

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A 25-year-old female presented with a generalized tonic-clonic seizure. She had no previous history of seizures. A brain magnetic resonance imaging scan revealed a solitary enhancing mass in the right fronto-parietal cortex. During surgery, the mass was noted to be pure cortical with no connection to the ventricular lining. The tumor was completely resected. After surgery, the patient had no further seizures. The biopsy result showed a supratentorial ependymoma, which was C11orf95-RELA -fusionpositive.

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Supratentorial cortical ependymoma: A systematic literature review and case illustration

et al. 2022

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Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized C11orf95-RELA fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with C11orf95- RELA fusions and supratentorial ependymomas with C11orf95 RELA fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of RELA fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with C11orf95- RELA fusions should be classified as a distinct entity.

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Cortically based cystic supratentorial RELA fusion-positive ependymoma: a case report with unusual presentation and appearance and review of literature

Cited by 4 publications

References 27 publications

Molecular Classification and Therapeutic Targets in Ependymoma

Molecular Classification and Therapeutic Targets in Ependymoma

Pure Cortical Ependymoma With RELA-Fusion Positive in a Young Adult

Supratentorial cortical ependymoma: A systematic literature review and case illustration

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