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Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature

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Abstract

Dysembryoplastic neuroepithelial tumors (DNETs) have traditionally been viewed as benign “quasihamartomatous” tumors widely considered curable with surgery alone. More recently, case reports have described malignant gliomas arising after irradiation and recurrences following subtotal or even gross total resection. Herein, we describe five cases of DNET with recurrences 2–7 years after resection. Although the radiology was often alarming (e.g., new ring enhancing mass), the pathology remained benign in most cases. Nonetheless, a probably radiation induced anaplastic astrocytoma was encountered in one case 7 years after therapy. These findings suggest that these patients may need closer follow-up than initially suggested, lending further support to the notion that this tumor behaves more like a benign neoplasm, rather than a dysplastic or hamartomatous lesion.

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Acknowledgments

The authors are grateful to Dr. Edith Schmidt from the Sunrise Hospital in Las Vegas (currently at the Children’s National Medical Center in Washington, DC) for sharing case #4 with us in consultation and providing additional clinical information.

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Authors and Affiliations

  1. Department of Neurological Surgery, Washington University School of Medicine, 660 S. Euclid Avenue, Campus Box 8057, St. Louis, MO, 63110, USA

    Wilson Z. Ray, Spiros L. Blackburn & Joshua L. Dowling

  2. Department of Pathology and Immunology, Division of Neuropathology, Washington University School of Medicine, 660 S. Euclid Avenue, Campus Box 8118, St. Louis, MO, 63110-1093, USA

    Arie Perry

  3. Department of Pathology, Instituto Nacional de Enfermedades Neoplasicas (INEN), Lima, Peru

    Sandro Casavilca-Zambrano & Carlos Barrionuevo

  4. Department of Neurosurgery, Instituto Nacional de Enfermedades Neoplasicas (INEN), Lima, Peru

    Jose E. Orrego & Hugo Heinicke

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  1. Wilson Z. Ray
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  7. Joshua L. Dowling
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Correspondence to Wilson Z. Ray.

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Ray, W.Z., Blackburn, S.L., Casavilca-Zambrano, S. et al. Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. J Neurooncol 94, 283–292 (2009). https://doi.org/10.1007/s11060-009-9849-9

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  • DOI: https://doi.org/10.1007/s11060-009-9849-9

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