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Big Cell Ependymoma of Lateral Ventricle: Case Report, Literature Assessment, and Evaluation of Prognostic Elements and Genetic Profile.

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Big Cell Ependymoma of Lateral Ventricle: Case Report, Literature Assessment, and Evaluation of Prognostic Elements and Genetic Profile.

World Neurosurg. 2017 Dec;108:997.e9-997.e14

Authors: Takami H, Graffeo CS, Perry A, Raghunathan A, Jenkins RB, Giannini C, Burns TC

BACKGROUND: Big cell ependymoma (GCE) is a uncommon major central nervous system neoplasm. We report a case of GCE arising within the lateral ventricle.
CASE DESCRIPTION: A 22-year-old feminine introduced with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered within the atrium of the fitting lateral ventricle with extension all through the frontal and temporal horns. An preliminary subtotal resection yielded the signature biphasic sample of GCE. The dominant element contained pleomorphic, bizarre-appearing large cells with low mitotic index, and a minor element comprised monomorphic, extremely mobile, mitotically energetic cells that shaped perivascular pseudorosettes. Array-comparative genomic hybridization confirmed copy quantity abnormalities in keeping with chromosomal instability with out proof of RELA- or YAP1-fusion-features most frequently seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgical procedure was undertaken to attenuate residual earlier than proton beam radiotherapy.
LITERATURE REVIEW: Assessment of the literature recognized 28 reported circumstances, with a median age of 34 and bimodal peaks at roughly 20 and 50 years of age, together with 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial circumstances concerned the fourth ventricle; no circumstances arose from the third or lateral ventricles. Supratentorial tumors predominated in youthful sufferers, whereas different areas have been noticed amongst older sufferers (21.6 vs. 46.three years of age; P = zero.01). Circumstances with Ki-67 index ?10% confirmed worse progression-free survival than these of <10% (P = zero.049).
CONCLUSION: Though uncommon, GCE must be thought-about within the differential of younger sufferers with atypical intraventricular lesions, notably provided that extent of resection is related to elevated survival and GCE is regarded as radiation resistant.

PMID: 28943417 [PubMed – indexed for MEDLINE]

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