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
Review
. 2023 Nov 20;15(11):e49086.
doi: 10.7759/cureus.49086. eCollection 2023 Nov.

Spinal Ependymomas: An Updated WHO Classification and a Narrative Review

Eliezer Villanueva-Castro  1 , Juan Marcos Meraz-Soto  1 , Itzel Ariadna Hernández-Dehesa  2 , Martha Lilia Tena-Suck  3 , Rebeca Hernández-Reséndiz  4 , Edgardo de Jesus Mateo-Nouel  1 , Juan Antonio Ponce-Gómez  1 , Juan Nicasio Arriada-Mendicoa  1
Affiliations
Review

Spinal Ependymomas: An Updated WHO Classification and a Narrative Review

Eliezer Villanueva-Castro et al. Cureus. .

Abstract

Ependymomas are neuroepithelial tumors that develop from ependymal cells found in the brain parenchyma and can spread to any part of the spinal cord. Three to six percent of all malignancies affecting the central nervous system (CNS) are ependymomas. Even the most talented surgeons are challenged by spinal cord ependymomas; as a result, research into this clinical phenomenon should continue. Since 1979, the World Health Organization (WHO) has published a classification and grading system for CNS malignancies to ensure consistent diagnostic standards worldwide. The WHO prepared an update on these tumors, paying particular attention to molecular techniques to categorize the therapeutic management of each patient with greater accuracy and clarity. We thoroughly reviewed the literature on the epidemiology, etiology, diagnosis, and treatment of spinal ependymomas since there has not been a recent review of these tumors. This included modifications to the 2021 WHO Classification of Tumors of the Central Nervous System.

Keywords: central nervous system tumors; ependymomas; literature review; neuro-oncology surgery; spinal ependymoma.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Search strategy
Figure 2
Figure 2. Image-based differential diagnosis
(A) Magnetic resonance imaging, T1 sagittal: a thoracic intradural and intramedullary lesion with a hypervascular nodule focused on the pial surface of the spinal cord, as well as syringomyelia and medullary edema with cranial and caudal extension, and the hypervascular nodule with homogeneous and avid uptake after gadolinium administration (yellow arrow). Histological diagnosis: hemangioblastoma. (B) Magnetic resonance imaging, T2 sagittal: intradural and intramedullary cervical lesion with poorly defined margins, homogeneous and hyperintense appearance, infiltrating and expansive features (yellow arrow). Histological diagnosis: astrocytoma. (C) Magnetic resonance imaging, T2 sagittal: intradural and intramedullary cervical lesion, with solid and cystic components, leading to fusiform enlargement of the spinal cord; solid component of RM behavior (yellow arrow). Histological diagnosis: cervical ependymoma.
Figure 3
Figure 3. Thoraco-lumbar spinal ependymoma
Intradural and intramedullary lesion with an oval shape and well-defined margins that enlarges and deforms the spinal canal at T10-L2. (A) Sagittal T1 hypointense, (B) sagittal T2 hyperintense, (C) sagittal STIR with intense and heterogeneous gadolinium enhancement and (D) axial T2 at L2 height with centripetal placement. Histological diagnosis: spinal ependymoma STIR, short tau inversion recovery
Figure 4
Figure 4. Histomorphology of ependymomas
(A-F) Small hyperchromatic cells with insufficient cytoplasm produce rosette and/or pseudorosette formations surrounding vessels. (C) The cells are hyperchromatic, the vasculature has a fibrillar shape, the cells may have distinct cellular atypia, the stroma between the cells is typically fibrillar, and apparent atypia can be seen depending on the histological grade.
Figure 5
Figure 5. Histomorphology of myxopapillary ependymomas
(A) Solid regions with flexible stroma, (B) papillary areas, (C) hyalinized vessels, (D) few cells with thicker hyalinized vessels.
See this image and copyright information in PMC

Similar articles

  • Molecular characterization of histopathological ependymoma variants.
    Neumann JE, Spohn M, Obrecht D, Mynarek M, Thomas C, Hasselblatt M, Dorostkar MM, Wefers AK, Frank S, Monoranu CM, Koch A, Witt H, Kool M, Pajtler KW, Rutkowski S, Glatzel M, Schüller U. Neumann JE, et al. Acta Neuropathol. 2020 Feb;139(2):305-318. doi: 10.1007/s00401-019-02090-0. Epub 2019 Nov 2. Acta Neuropathol. 2020. PMID: 31679042
  • Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors.
    Fukuoka K, Kanemura Y, Shofuda T, Fukushima S, Yamashita S, Narushima D, Kato M, Honda-Kitahara M, Ichikawa H, Kohno T, Sasaki A, Hirato J, Hirose T, Komori T, Satomi K, Yoshida A, Yamasaki K, Nakano Y, Takada A, Nakamura T, Takami H, Matsushita Y, Suzuki T, Nakamura H, Makino K, Sonoda Y, Saito R, Tominaga T, Matsusaka Y, Kobayashi K, Nagane M, Furuta T, Nakada M, Narita Y, Hirose Y, Ohba S, Wada A, Shimizu K, Kurozumi K, Date I, Fukai J, Miyairi Y, Kagawa N, Kawamura A, Yoshida M, Nishida N, Wataya T, Yamaoka M, Tsuyuguchi N, Uda T, Takahashi M, Nakano Y, Akai T, Izumoto S, Nonaka M, Yoshifuji K, Kodama Y, Mano M, Ozawa T, Ramaswamy V, Taylor MD, Ushijima T, Shibui S, Yamasaki M, Arai H, Sakamoto H, Nishikawa R, Ichimura K; Japan Pediatric Molecular Neuro-Oncology Group (JPMNG). Fukuoka K, et al. Acta Neuropathol Commun. 2018 Dec 4;6(1):134. doi: 10.1186/s40478-018-0630-1. Acta Neuropathol Commun. 2018. PMID: 30514397 Free PMC article.
  • Recent Advances in the Classification and Treatment of Ependymomas.
    Leeper H, Felicella MM, Walbert T. Leeper H, et al. Curr Treat Options Oncol. 2017 Aug 10;18(9):55. doi: 10.1007/s11864-017-0496-7. Curr Treat Options Oncol. 2017. PMID: 28795287 Review.
  • Classification and neuroimaging of ependymal tumors.
    Mu W, Dahmoush H. Mu W, et al. Front Pediatr. 2023 May 23;11:1181211. doi: 10.3389/fped.2023.1181211. eCollection 2023. Front Pediatr. 2023. PMID: 37287627 Free PMC article. Review.
  • Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging.
    Pagès M, Pajtler KW, Puget S, Castel D, Boddaert N, Tauziède-Espariat A, Picot S, Debily MA, Kool M, Capper D, Sainte-Rose C, Chrétien F, Pfister SM, Pietsch T, Grill J, Varlet P, Andreiuolo F. Pagès M, et al. Brain Pathol. 2019 May;29(3):325-335. doi: 10.1111/bpa.12664. Epub 2018 Nov 28. Brain Pathol. 2019. PMID: 30325077 Free PMC article.
See all similar articles

Cited by

References

    1. The biology of ependymomas and emerging novel therapies. Saleh AH, Samuel N, Juraschka K, Saleh MH, Taylor MD, Fehlings MG. Nat Rev Cancer. 2022;22:208–222. - PubMed
    1. The 2021 WHO Classification of Tumors of the Central Nervous System: clinical implications. Wen PY, Packer RJ. Neuro Oncol. 2021;23:1215–1217. - PMC - PubMed
    1. Neuroimaging of spine tumors. Pinter NK, Pfiffner TJ, Mechtler LL. Handb Clin Neurol. 2016;136:689–706. - PubMed
    1. cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Ellison DW, Aldape KD, Capper D, et al. Brain Pathol. 2020;30:863–866. - PMC - PubMed
    1. Epidemiology, molecular classification and WHO grading of ependymoma. Hübner JM, Kool M, Pfister SM, Pajtler KW. J Neurosurg Sci. 2018;62:46–50. - PubMed

LinkOut - more resources