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      Intradural extramedullary double primary ependymoma and meningioma rare condition: Case report and literature review

      research-article
      , MASc a , , MASc b , , MASc a , * ,
      Medicine
      Lippincott Williams & Wilkins
      ependymoma, intradural extramedullary, meningioma

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          Abstract

          Rationale:

          Ependymomas are commonly prevalent intramedullary neoplasms in adults, with hardly any cases of exophytic extramedullary ependymoma being reported. Meningiomas, on the contrary, are one of the most common intradural extramedullary (IDEM) tumors. However, the occurrence of both IDEM tumors simultaneously is extremely rare.

          Patient concerns:

          A 63-year-old female who presented with pain and numbness in both lower limbs, and symptoms rapidly progressed over the past 5 months.

          Diagnosis:

          Based on the patient’s clinical symptoms and imaging features, we conducted pathological examination and genetic testing, ultimately confirming that the patient had IDEM double primary ependymoma and meningioma.

          Interventions:

          Surgery was performed to remove double spinal tumors, decompress spinal nerve roots, and perform laminectomy, and she was treated with electrocardiogram monitoring, antibiotics, hemostasis, and antiedema therapy.

          Outcomes:

          Histopathology confirmed World Health Organization grade II ependymoma at L2 and World Health Organization grade I meningioma at T12-L1. MYCN amplification and other genetic alterations were absent. Postoperative recovery was favorable, with no recurrence at 6-month follow-up.

          Lessons:

          This is the first reported case of IDEM double primary ependymoma and meningioma, highlighting the rarity of such cases and the importance of thorough diagnostic workup and surgical excision for IDEM tumors. Genetic analysis adds to the understanding of these rare tumors and guides management strategies.

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          Most cited references31

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          The 2021 WHO Classification of Tumors of the Central Nervous System: a summary

          The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors. Building on the 2016 updated fourth edition and the work of the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy, the 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification. At the same time, it remains wedded to other established approaches to tumor diagnosis such as histology and immunohistochemistry. In doing so, the fifth edition establishes some different approaches to both CNS tumor nomenclature and grading and it emphasizes the importance of integrated diagnoses and layered reports. New tumor types and subtypes are introduced, some based on novel diagnostic technologies such as DNA methylome profiling. The present review summarizes the major general changes in the 2021 fifth edition classification and the specific changes in each taxonomic category. It is hoped that this summary provides an overview to facilitate more in-depth exploration of the entire fifth edition of the WHO Classification of Tumors of the Central Nervous System.
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            Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.

            Ependymal tumors across age groups are currently classified and graded solely by histopathology. It is, however, commonly accepted that this classification scheme has limited clinical utility based on its lack of reproducibility in predicting patients' outcome. We aimed at establishing a uniform molecular classification using DNA methylation profiling. Nine molecular subgroups were identified in a large cohort of 500 tumors, 3 in each anatomical compartment of the CNS, spine, posterior fossa, supratentorial. Two supratentorial subgroups are characterized by prototypic fusion genes involving RELA and YAP1, respectively. Regarding clinical associations, the molecular classification proposed herein outperforms the current histopathological classification and thus might serve as a basis for the next World Health Organization classification of CNS tumors.
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              EANO guidelines for the diagnosis and treatment of meningiomas.

              Although meningiomas are the most common intracranial tumours, the level of evidence to provide recommendations for the diagnosis and treatment of meningiomas is low compared with other tumours such as high-grade gliomas. The meningioma task force of the European Association of Neuro-Oncology (EANO) assessed the scientific literature and composed a framework of the best possible evidence-based recommendations for health professionals. The provisional diagnosis of meningioma is mainly made by MRI. Definitive diagnosis, including histological classification, grading, and molecular profiling, requires a surgical procedure to obtain tumour tissue. Therefore, in many elderly patients, observation is the best therapeutic option. If therapy is deemed necessary, the standard treatment is gross total surgical resection including the involved dura. As an alternative, radiosurgery can be done for small tumours, or fractionated radiotherapy in large or previously treated tumours. Treatment concepts combining surgery and radiosurgery or fractionated radiotherapy, which enable treatment of the complete tumour volume with low morbidity, are being developed. Pharmacotherapy for meningiomas has remained largely experimental. However, antiangiogenic drugs, peptide receptor radionuclide therapy, and targeted agents are promising candidates for future pharmacological approaches to treat refractory meningiomas across all WHO grades.
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                Author and article information

                Contributors
                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MD
                Medicine
                Lippincott Williams & Wilkins (Hagerstown, MD )
                0025-7974
                1536-5964
                10 January 2025
                10 January 2025
                : 104
                : 2
                : e41210
                Affiliations
                [a ]Department of Pathology, Deyang Peoples’ Hospital, Deyang, Sichuan Province, China
                [b ]Department of Orthopaedics, Deyang Peoples’ Hospital, Deyang, Sichuan Province, China
                Author notes
                [* ]Correspondence: Qian Liu, Department of Pathology, Deyang Peoples’ Hospital, No. 173, section 3, North Taishan Road, Deyang, 618000, Sichuan Province, China (e-mail: liuqian_dy@ 123456163.com ).
                Author information
                https://orcid.org/0000-0002-8839-5187
                https://orcid.org/0000-0003-0616-4893
                Article
                MD-D-24-10124 00042
                10.1097/MD.0000000000041210
                11729158
                39792750
                b68d747c-2a62-4bfc-a8bb-aa0d590b5cbc
                Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 August 2024
                : 06 December 2024
                : 17 December 2024
                Categories
                5300
                Research Article
                Clinical Case Report
                Custom metadata
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                ependymoma,intradural extramedullary,meningioma
                ependymoma, intradural extramedullary, meningioma

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