Giant Cell Ependymoma of Lateral Ventricle: Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile

Hirokazu Takami, Christopher S. Graffeo, Avital Perry, Aditya Raghunathan, Robert Brian Jenkins, Caterina Giannini, Terence Burns

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. Case Description: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes. Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgery was undertaken to minimize residual before proton beam radiotherapy. Literature Review: Review of the literature identified 28 reported cases, with a median age of 34 and bimodal peaks at approximately 20 and 50 years of age, including 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial cases involved the fourth ventricle; no cases arose from the third or lateral ventricles. Supratentorial tumors predominated in younger patients, whereas other locations were observed among older patients (21.6 vs. 46.3 years of age; P = 0.01). Cases with Ki-67 index ≥10% showed worse progression-free survival than those of <10% (P = 0.049). Conclusion: Although rare, GCE should be considered in the differential of young patients with atypical intraventricular lesions, particularly given that extent of resection is associated with increased survival and GCE is thought to be radiation resistant.

Original languageEnglish (US)
JournalWorld Neurosurgery
DOIs
StateAccepted/In press - 2017

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Ependymoma
Lateral Ventricles
Giant Cells
Second-Look Surgery
Supratentorial Neoplasms
Fourth Ventricle
Central Nervous System Neoplasms
Chromosomal Instability
Mitotic Index
Third Ventricle
Comparative Genomic Hybridization
Radiation Tolerance
Temporal Lobe
Disease-Free Survival
Heart Ventricles
Protons
Seizures
Radiotherapy
Magnetic Resonance Imaging
Radiation

Keywords

  • Array-comparative genomic hybridization
  • Chromosomal instability
  • Giant cell ependymoma
  • Intraventricular tumors
  • Literature review
  • Rare diseases

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

@article{3342dd0329dc4c429d4e651fe14ba55b,
title = "Giant Cell Ependymoma of Lateral Ventricle: Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile",
abstract = "Background: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. Case Description: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes. Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgery was undertaken to minimize residual before proton beam radiotherapy. Literature Review: Review of the literature identified 28 reported cases, with a median age of 34 and bimodal peaks at approximately 20 and 50 years of age, including 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial cases involved the fourth ventricle; no cases arose from the third or lateral ventricles. Supratentorial tumors predominated in younger patients, whereas other locations were observed among older patients (21.6 vs. 46.3 years of age; P = 0.01). Cases with Ki-67 index ≥10{\%} showed worse progression-free survival than those of <10{\%} (P = 0.049). Conclusion: Although rare, GCE should be considered in the differential of young patients with atypical intraventricular lesions, particularly given that extent of resection is associated with increased survival and GCE is thought to be radiation resistant.",
keywords = "Array-comparative genomic hybridization, Chromosomal instability, Giant cell ependymoma, Intraventricular tumors, Literature review, Rare diseases",
author = "Hirokazu Takami and Graffeo, {Christopher S.} and Avital Perry and Aditya Raghunathan and Jenkins, {Robert Brian} and Caterina Giannini and Terence Burns",
year = "2017",
doi = "10.1016/j.wneu.2017.09.088",
language = "English (US)",
journal = "World Neurosurgery",
issn = "1878-8750",
publisher = "Elsevier Inc.",

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T1 - Giant Cell Ependymoma of Lateral Ventricle

T2 - Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile

AU - Takami, Hirokazu

AU - Graffeo, Christopher S.

AU - Perry, Avital

AU - Raghunathan, Aditya

AU - Jenkins, Robert Brian

AU - Giannini, Caterina

AU - Burns, Terence

PY - 2017

Y1 - 2017

N2 - Background: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. Case Description: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes. Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgery was undertaken to minimize residual before proton beam radiotherapy. Literature Review: Review of the literature identified 28 reported cases, with a median age of 34 and bimodal peaks at approximately 20 and 50 years of age, including 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial cases involved the fourth ventricle; no cases arose from the third or lateral ventricles. Supratentorial tumors predominated in younger patients, whereas other locations were observed among older patients (21.6 vs. 46.3 years of age; P = 0.01). Cases with Ki-67 index ≥10% showed worse progression-free survival than those of <10% (P = 0.049). Conclusion: Although rare, GCE should be considered in the differential of young patients with atypical intraventricular lesions, particularly given that extent of resection is associated with increased survival and GCE is thought to be radiation resistant.

AB - Background: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. Case Description: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes. Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgery was undertaken to minimize residual before proton beam radiotherapy. Literature Review: Review of the literature identified 28 reported cases, with a median age of 34 and bimodal peaks at approximately 20 and 50 years of age, including 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial cases involved the fourth ventricle; no cases arose from the third or lateral ventricles. Supratentorial tumors predominated in younger patients, whereas other locations were observed among older patients (21.6 vs. 46.3 years of age; P = 0.01). Cases with Ki-67 index ≥10% showed worse progression-free survival than those of <10% (P = 0.049). Conclusion: Although rare, GCE should be considered in the differential of young patients with atypical intraventricular lesions, particularly given that extent of resection is associated with increased survival and GCE is thought to be radiation resistant.

KW - Array-comparative genomic hybridization

KW - Chromosomal instability

KW - Giant cell ependymoma

KW - Intraventricular tumors

KW - Literature review

KW - Rare diseases

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