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Systematic Review of Clinical, Radiologic, and Histologic Features of Benign Notochordal Cell Tumors: Implications for Patient Management

Usher, I; Flanagan, AM; Choi, D; (2019) Systematic Review of Clinical, Radiologic, and Histologic Features of Benign Notochordal Cell Tumors: Implications for Patient Management. World Neurosurgery , 130 pp. 13-23. 10.1016/j.wneu.2019.06.009. Green open access

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Abstract

BACKGROUND: There are no absolute defining criteria for benign notochordal cell tumors; the diagnosis is usually based on small size and the absence of aggressive features. Therefore, by definition, the diagnosis is subjective and usually determined by multidisciplinary consensus. A benign notochordal cell tumor should not grow during surveillance, and this may be used to confirm the diagnosis, but is a tautologic definition. Diagnostic ambiguity leads to uncertainty in management. If a tumor is a small chordoma then early surgery is likely to provide a better outcome. However, unnecessary treatment of a benign tumor may incur unjustified risk. OBJECTIVE: To propose clearer guidelines for the definition and management of benign notochordal tumors. METHODS: We performed a PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) review of the reported definitions for benign notochordal tumors and their management. RESULTS: The accepted features of benign notochordal tumors vary considerably: a typical tumor may be diagnosed in the absence of neurology, radiologically well-corticated bony margins, size <35 mm, no enhancement with contrast, no soft tissue extension, no dural penetration, no progression on scans, histologic absence of extracellular myxoid matrix, and low Ki67 index. If these criteria are fulfilled, it is reasonable to use radiologic surveillance in the first instance. Biopsy may be offered depending on the relative risks of performing the biopsy, or if there are atypical features. CONCLUSIONS: We suggest a clearer definition for a benign notochordal tumor and a management algorithm that incorporates a level of diagnostic uncertainty.

Type: Article
Title: Systematic Review of Clinical, Radiologic, and Histologic Features of Benign Notochordal Cell Tumors: Implications for Patient Management
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.wneu.2019.06.009
Publisher version: https://doi.org/10.1016/j.wneu.2019.06.009
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Bone neoplasms, Chordoma, Notochord/pathology, Spinal neoplasms/pathology
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Brain Repair and Rehabilitation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Pathology
URI: https://discovery.ucl.ac.uk/id/eprint/10080688
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