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Low-grade ependymoma with late metastasis: autopsy case study and literature review

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Abstract

Introduction

Ependymoma metastasis occurs usually along with local recurrence within 7 years after the initial diagnosis. Later spinal metastasis without local recurrence after the surgical resection has been rarely reported in patients with low-grade ependymomas but not with high-grade ependymomas. Here, we present a case with autopsy revealing late extensive supratentorial metastasis of a fourth ventricle classic WHO grade II ependymoma with no local recurrence or spinal metastasis.

Methods

A 4-year-old boy underwent a gross total resection (GTR) of the fourth ventricle ependymoma and postoperative radiation therapy. Follow-up MRI showed no recurrence for the next 7 years, but a half year later, extra-axial tumors in the left cerebellopontine angle and right frontal lobe were observed. GTR of the left cerebellopontine angle ependymoma was performed, followed by additional radiation therapy.

Results

He was stable for the following 2 years before MRI revealed growth of the right frontal tumor and new lesions. GTR of the right frontal tumor demonstrated similar pathologic features of ependymoma. Despite chemotherapy, follow-up MRIs exhibited increasing numbers and sizes of supratentorial tumors but no infratentorial or spinal tumors. He died 15 years after the initial diagnosis. Postmortem brain examination confirmed the supratentorial subarachnoid dissemination with multifocal metastases of classic ependymomas but no recurrence at the infratentorial sites.

Conclusion

Our case study and literature review suggest that low-grade ependymomas under the current WHO classification have the risk of late metastasis. Therefore, long-term follow-up of the whole neuroaxis is more important for the patients with low-grade ependymomas even in the absence of local recurrence.

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Acknowledgments

The authors thank Drs. Clarissa Fauth, Sushil Parai, and Consolato Sergi for their help in collecting the brain for this postmortem neuropathologic study and Ms. Clare McDonald for her help in providing clinical information.

Conflict of interest

The authors are not receiving any financial or material support in conjunction with the generation of this submission. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.

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Authors and Affiliations

  1. Department of Surgery, University of Alberta, 8440-112 Street, Edmonton, AB, T6G 2B7, Canada

    Wael Alshaya, Vivek Mehta & Keith E. Aronyk

  2. Department of Pediatrics, University of Alberta, 8440-112 Street, Edmonton, AB, T6G 2B7, Canada

    Beverly A. Wilson

  3. Department of Oncology, University of Alberta, 8440-112 Street, Edmonton, AB, T6G 2B7, Canada

    Susan Chafe

  4. Department of Laboratory Medicine and Pathology, University of Alberta, 8440-112 Street, Edmonton, AB, T6G 2B7, Canada

    Jian-Qiang Lu

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  1. Wael Alshaya
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  2. Vivek Mehta
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  3. Beverly A. Wilson
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  4. Susan Chafe
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  5. Keith E. Aronyk
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Correspondence to Jian-Qiang Lu.

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Alshaya, W., Mehta, V., Wilson, B.A. et al. Low-grade ependymoma with late metastasis: autopsy case study and literature review. Childs Nerv Syst 31, 1565–1572 (2015). https://doi.org/10.1007/s00381-015-2744-0

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  • DOI: https://doi.org/10.1007/s00381-015-2744-0

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