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Imaging features to distinguish posterior fossa ependymoma subgroups

  • Paediatric
  • Published:
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Abstract

Objectives

Posterior fossa ependymoma group A (EPN_PFA) and group B (EPN_PFB) can be distinguished by their DNA methylation and give rise to different prognoses. We compared the MRI characteristics of EPN_PFA and EPN_PFB at presentation.

Methods

Preoperative imaging of 68 patients with posterior fossa ependymoma from two centers was reviewed by three independent readers, blinded for histomolecular grouping. Location, tumor extension, tumor volume, hydrocephalus, calcifications, tissue component, enhancement or diffusion signal, and histopathological data (cellular density, calcifications, necrosis, mitoses, vascularization, and microvascular proliferation) were compared between the groups. Categorical data were compared between groups using Fisher’s exact tests, and quantitative data using Mann–Whitney tests. We performed a Benjamini–Hochberg correction of the p values to account for multiple tests.

Results

Fifty-six patients were categorized as EPN_PFA and 12 as EPN_PFB, with median ages of 2 and 20 years, respectively (p = 0.0008). The median EPN_PFA tumoral volume was larger (57 vs 29 cm3, p = 0.003), with more pronounced hydrocephalus (p = 0.002). EPN_PFA showed an exclusive central position within the 4th ventricle in 61% of patients vs 92% for EPN_PFB (p = 0.01). Intratumor calcifications were found in 93% of EPN_PFA vs 40% of EPN_PFB (p = 0.001). Invasion of the posterior fossa foramina was mostly found for EPN_PFA, particularly the foramina of Luschka (p = 0.0008). EPN_PFA showed whole and homogeneous tumor enhancement in 5% vs 75% of EPN_PFB (p = 0.0008). All mainly cystic tumors were EPN_PFB (p = 0.002). The minimal and maximal relative ADC was slightly lower in EPN_PFA (p = 0.02 and p = 0.01, respectively).

Conclusion

Morphological characteristics from imaging differ between posterior fossa ependymoma subtypes and may help to distinguish them preoperatively.

Clinical relevance statement

This study provides a tool to differentiate between group A and group B ependymomas, which will ultimately allow the therapeutic strategy to be adapted in the early stages of patient management.

Key Points

• Posterior fossa ependymoma subtypes often have different imaging characteristics.

• Posterior fossa ependymomas group A are commonly median or lateral tissular calcified masses, with incomplete enhancement, affecting young children and responsible for pronounced hydrocephalus and invasion of the posterior fossa foramina.

• Posterior fossa ependymomas group B are commonly median non-calcified masses of adolescents and adults, predominantly cystic, and minimally invasive, with total and homogeneous enhancement.

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Abbreviations

CBF:

Cerebral blood flow

CNS:

Central nervous system

EPN_PF:

Posterior fossa ependymoma

EPN_PFA:

Posterior fossa ependymoma group A

EPN_PFB:

Posterior fossa ependymoma group B

IQR:

Interquartile range

WHO:

World Health Organization

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Funding

The authors state that this work has not received any funding.

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Authors and Affiliations

  1. Pediatric Radiology Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Hôpital Universitaire Necker-Enfants Malades, 149 Rue de Sèvres, 75015, Paris, France

    Thomas Leclerc, Raphael Levy, Charles-Joris Roux, Nathalie Boddaert & Volodia Dangouloff-Ros

  2. Université Paris Cité, INSERM U1299, Paris, France

    Thomas Leclerc, Raphael Levy, Charles-Joris Roux, Nathalie Boddaert & Volodia Dangouloff-Ros

  3. UMR 1163, Institut Imagine, Université Paris Cité, Paris, France

    Thomas Leclerc, Raphael Levy, Charles-Joris Roux, Nathalie Boddaert & Volodia Dangouloff-Ros

  4. Neuropathology Department, GHU Paris, Université Paris Cité, Paris, France

    Arnault Tauziède-Espariat & Pascale Varlet

  5. Pediatric Neurosurgery Department, AP-HP, Hôpital Universitaire Necker-Enfants Malades, Paris, France

    Kevin Beccaria, Thomas Blauwblomme & Stéphanie Puget

  6. Université Paris Cité, Paris, France

    Kevin Beccaria & Thomas Blauwblomme

  7. Department of Pediatric and Adolescent Oncology, Gustave Roussy Institute, Villejuif, France

    Jacques Grill, Christelle Dufour, Léa Guerrini-Rousseau & Samuel Abbou

  8. Department of Radiotherapy Oncology, Gustave Roussy, Villejuif, France

    Stéphanie Bolle

  9. Neurosurgery Department, GHU Paris, Université Paris Cité, Paris, France

    Alexandre Roux & Johan Pallud

  10. Neuroradiology Department, GHU Paris, Université Paris Cité, Paris, France

    Corentin Provost & Catherine Oppenheim

  11. INSERM U1266, Institut de Psychiatrie Et Neurosciences de Paris, Université Paris Cité, Paris, France

    Corentin Provost, Catherine Oppenheim & Pascale Varlet

Authors
  1. Thomas Leclerc
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  2. Raphael Levy
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  3. Arnault Tauziède-Espariat
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  16. Catherine Oppenheim
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  19. Volodia Dangouloff-Ros
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Corresponding author

Correspondence to Volodia Dangouloff-Ros.

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Guarantor

The scientific guarantor of this publication is Volodia Dangouloff-Ros.

Conflict of interest

The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Statistics and biometry

One of the authors has significant statistical expertise.

Informed consent

Written informed consent was obtained from all subjects (patients) in this study.

Ethical approval

Ethical committee approval was obtained to study the multimodal imaging of the children’s brain tumors (EDRACT 2014-A-00541–46).

Study subjects or cohorts overlap

None.

Methodology

• retrospective

• observational

• performed at two institutions

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Leclerc, T., Levy, R., Tauziède-Espariat, A. et al. Imaging features to distinguish posterior fossa ependymoma subgroups. Eur Radiol 34, 1534–1544 (2024). https://doi.org/10.1007/s00330-023-10182-5

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  • DOI: https://doi.org/10.1007/s00330-023-10182-5

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