Available online 27 March 2024, 152299

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Our study details spinal ependymomas and myxopapillary ependymomas, highlighting their diversity and prognostic challenges.

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MYCN expression was uniformly absent, limiting its diagnostic or prognostic value.

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Despite favorable outcomes, recurrence underscores the need for further aggressiveness analysis.

AbstractBackground

Ependymomas (EPNs) of the spinal region are a heterogeneous group of tumors that account for 17.6 % in adults. Four types have been recognized: subependymoma, spinal ependymoma (Sp-EPN), myxopapillary ependymoma (MPE), and Sp-EPN-MYCN amplified, each with distinct histopathological and molecular features.

Methods

This study investigated the clinical and pathological characteristics and MYCN expression levels of 35 Sp-EPN and MPE cases diagnosed at a tertiary university hospital over a decade-long period.

Results

Twenty-five cases were Sp-EPN and 10 cases were MPE, and were graded as WHO grade 2, except for 1 Sp-EPN case with grade 3 features. The most common symptoms were lower back pain and difficulty in walking. Radiology showed different tumor sizes and locations along the spinal cord, with MPEs exclusively in the lumbosacral region. Surgery was the main treatment, and gross total resection was achieved in all cases except for one. Immunohistochemistry showed low Ki-67 proliferation indices in all cases, and no MYCN expression. During follow-up, 3 (8.6 %) cases recurred and/or metastasized and 5 cases (14.3 %) died. No significant difference was found in disease-free survival or overall survival between Sp-EPN and MPE cases. However, 3 cases with grade 2 histology demonstrated recurrence and/or metastasis, despite the lack of MYCN expression.

Conclusion

Our results underscore the multifactorial nature of tumor aggressiveness in EPNs of the spinal region. This study enhances our knowledge of the clinical and pathological features of Sp-EPNs and MPEs and highlights the need for better diagnostic and prognostic markers in these rare tumors.

Section snippetsBackground

Ependymal tumors account for 20.6 % of primary spinal tumors in children and adolescents, and 17.6 % in adults over the age of 20, as reported by the Central Brain Tumor Registry of the United States [1]. The median age at diagnosis for patients with spinal EPN varies from 25 to 45 years across different studies. They represent the most frequent primary tumors of the spinal cord, with a male-to-female ratio ranging from 1:1.3 to 2.16:1 [2]. EPNs constitute a heterogeneous tumor group, ranging

Study approval

Ethical approval was obtained from the Non-Interventional Clinical Research Ethics Committee of XXX University XXX Hospital, with decision number 973 dated September 2020.

1.2.

Clinical and Pathological Information:

Ependymomas of the spinal cord that were diagnosed between February 2009 and February 2020 at the Pathology Department of XXX University XXX Hospital were investigated. Data including age, gender, tumor location, tumor size, histological grade according to the 2021 WHO classification of

Results3.1.

Patients and Clinical Characteristics

Typing the keywords “ependymoma,” “spinal,” and “vertebra” in the hospital information system, a total of 47 patients with EPN diagnosis were identified. One case with subependymoma diagnosis was not included in the study. Eight cases located outside the spinal region and 3 cases with missing blocks were excluded. A total of 35 patients were included in the study. There were 21 females (60 %) and 14 males (40 %). The median age of the patients was 43 years

Discussion

According to the 2021 WHO CNS tumor classification, EPNs of the spinal region encompass various types, including Sp-EPN, Sp-EPN-MYCN, MPE, and subependymoma, each characterized by a combination of histopathological findings, molecular features, and anatomical site. Sp-EPN-MYCN has been adopted with 27 identified cases, most of which have high-grade (grade 3) histopathological features and are described as an aggressive tumor with poor outcome compared to other Sp-EPN cases [3]. In our study, we

Ethics approval and consent to participate

This study was approved by the Institutional Review Board at XXX University, XXX Hospital with a decision number 973 dated September 2020.

Consent for publication

The patients have given consent to have this manuscript published in an academic journal.

Funding

Financial support was received from the Directorate of Revolving Fund of XXX University XXX Hospital for immunohistochemical staining studies.

CRediT authorship contribution statement

Fikret Dirilenoğlu: Writing – review & editing, Writing – original draft, Visualization, Investigation. Gözde Topel: Writing – review & editing, Writing – original draft, Methodology, Funding acquisition, Formal analysis, Data curation, Conceptualization. İsmail Ertan Sevin: Writing – review & editing, Investigation, Formal analysis, Data curation. Aslı Kahraman: Writing – review & editing, Validation, Supervision, Methodology, Funding acquisition, Data curation, Conceptualization.

Declaration of competing interest

The authors declare that they have no competing interests.

Acknowledgements

This study was derived from a thesis project, completed in September 2022.

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