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Extradural malignant rhabdoid tumor of the spine in children: A case-based review

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Abstract

Background

Extradural malignant rhabdoid tumors of the spine are highly malignant and invasive tumors (WHO grade IV) with poor prognosis, most frequently occurring in young children before 2 years of age. Pain and motor deficit are the most common presenting signs.

Case description

We report a case of a 2-year-old girl presenting with axial ataxia and paraparesis related to an extradural malignant rhabdoid tumor causing posterior thoracic spinal cord compression (D3–D6). She underwent two near-total removal of the tumor, adjuvant chemotherapy according to the Eu-Rhab protocol and proton beam therapy. She then developed multiple cranial nerve paresis (meningeal carcinomatosis) after 4 cycles of chemotherapy and died at 4.32 months of follow-up.

Discussion and conclusion

The role of the PET scan was essential to guide us to remove a residue, while two concomitant spinal MRIs were considered negative. We reviewed the 16 cases reported in the literature. Multiple surgeries and radiotherapy seem to be correlated with longer survival. No child younger than 2 years old had a documented survival higher than 4.32 months.

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Availability of data and material

All medical data are available and recorded in a secure computerized medical file.

Code availability

Not applicable.

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Funding

No financial support was received for this work.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium

    Eric Vigneul, Herbert Rooijakkers, Vincent Joris & Christian Raftopoulos

  2. Laboratory of Neural Differentiation (NEDI), Animal Molecular and Cellular Biology Group, Louvain Institute of Biomolecular Science and Technology, Université catholique de Louvain, Louvain-la-Neuve, Belgium

    Eric Vigneul

  3. Anatomic Pathology Department, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium

    Lina Daoud

  4. Department of Pediatric Hematology and Oncology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium

    An Van Damme

Authors
  1. Eric Vigneul
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  2. Herbert Rooijakkers
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  3. Vincent Joris
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  4. Lina Daoud
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  5. An Van Damme
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  6. Christian Raftopoulos
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Contributions

EV: conception of the draft, conception of the figures and tables, clinical management of the patient. HR: substantial contributions to the conception of the work, clinical management of the patient. VJ: substantial contributions to the conception and design of the work, clinical management of the patient. LD: substantial contributions to the conception and design of the work, conception of the figures. AVD: conception of the draft, substantial contributions to the conception and design of the work, clinical management of the patient. CR: substantial contributions to the conception and design of the work.

Corresponding author

Correspondence to Christian Raftopoulos.

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The patient’s family has consented to the submission of the case report for submission to the journal.

Conflict of interest

The authors declare no competing interests.

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The manuscript is an original contribution, not submitted simultaneously in another journal or published elsewhere, partially or in full in any form or language. Results are presented clearly, honestly, and without fabrication, falsification or inappropriate data manipulation (including image based manipulation). Authors adhere to discipline-specific rules for acquiring, selecting and processing data.

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Vigneul, E., Rooijakkers, H., Joris, V. et al. Extradural malignant rhabdoid tumor of the spine in children: A case-based review. Childs Nerv Syst 40, 979–986 (2024). https://doi.org/10.1007/s00381-023-06224-4

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  • DOI: https://doi.org/10.1007/s00381-023-06224-4

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