Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2023 Oct;11(4):281-288.
doi: 10.14791/btrt.2023.0030.

Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures

Changjun Hyun #  1 , Yeonju Lee #  1 , Ho Kang  2 , Hyun Joo Park  2 , Koung Jin Suh  3 , Byung Se Choi  4 , Gheeyoung Choe  5 , Chae-Yong Kim  6
Affiliations
Case Reports

Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures

Changjun Hyun et al. Brain Tumor Res Treat. 2023 Oct.

Abstract

Ewing sarcoma and peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant tumor that is most prevalent in children and young adults and often radiologically mimics a meningioma. A 38-year-old female patient visited our hospital with complaints of right-sided tinnitus, right hemiparesis, and imbalance. She underwent preoperative imaging and was subsequently diagnosed as having a meningioma on the petrous ridge. After partial resection, EWSR1-FLI1 gene fusion was confirmed, and she was diagnosed with ES/pPNET. The tumor was successfully treated using a multidisciplinary approach of adjuvant chemo- and radiotherapy. This case is noteworthy because it is an extremely rare case of an intracranial ES/pPNET, and it is worth sharing our clinical experience that the tumor was successfully treated through a multidisciplinary therapeutic approach even though complete resection was not achieved.

Keywords: EWSR1; Ewing sarcoma; FLI1; Meningioma; Peripheral primitive neuroectodermal tumor.

PubMed Disclaimer

Conflict of interest statement

Chae-Yong Kim, a contributing editor of Brain Tumor Research and Treatment, was not involved in the editorial evaluation or decision to publish this article. All remaining authors have declared no conflicts of interest.

Figures

Fig. 1
Fig. 1. Preoperative images of the patient: T1-weighted image (A), T2-weighted image (B), contrast-enhanced T1-weighted image (C), ADC map (D), and CBV map (E) of MRI; and 18F-FDG PET/CT (F). ADC, apparent diffusion coefficient; CBV, cerebral blood volume; FDG, fluorodeoxyglucose; PET/CT, positron emission tomography/computed tomography.
Fig. 2
Fig. 2. Pathologic results of the patient. Tissue stained by hematoxylin and eosin (A), immunohistochemically stained by GFAP (B), VMA (C), CD99 (D), and vimentin (E), and tissue with fluorescence in situ hybridization using dual-color break-apart rearrangement probes which flank the EWSR1 breakpoint (F). Pseudorosette formation and rearranged chromosome 22 are annotated by white triangles (A) and arrows (B), respectively. GFAP, glial fibrillary acidic protein; VMA, vimentin antibodies; CD, cluster of differentiation.
Fig. 3
Fig. 3. Postoperative images of the patient: T2-weighted image at POD 15 (A), CT at POD 31 (B), contrast-enhanced T1-weighted image 3 months after surgery (C), and contrast-enhanced T1-weighted image 10 months after surgery (D). POD, postoperative day.
See this image and copyright information in PMC

References

    1. Chen J, Cheng R, Fan F, Zheng Y, Li Y, Chen Y, et al. Cranial Ewing sarcoma/peripheral primitive neuroectodermal tumors: a retrospective study focused on prognostic factors and long-term outcomes. Front Oncol. 2019;9:1023. - PMC - PubMed
    1. Deshpande G, Epari S, Gupta C, Shetty O, Gurav M, Chinnaswamy G, et al. Primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases. Childs Nerv Syst. 2021;37:839–849. - PubMed
    1. Geng Z, Gao W, Cheng W, Wu A. Primary intracranial Ewing sarcoma invading the superior sagittal sinus with EWSR1-FLI1 gene fusion and EWSR1 gene mutation: a case report and literature review. World Neurosurg. 2023;175:1–10. - PubMed
    1. Jiang Y, Zhao L, Wang Y, Liu X, Wu X, Li Y. Primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking meningioma: a case report and literature review. Front Oncol. 2020;10:528073. - PMC - PubMed
    1. Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, et al. Primary intracranial pPNET/Ewing sarcoma: diagnosis, management, and prognostic factors dilemma–a systematic review of the literature. World Neurosurg. 2018;115:346–356. - PubMed

Publication types