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Historical Overview, Demographics, and Clinical Presentation of Spinal Chordoma

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Chordoma of the Spine

Abstract

Chordomas are slow-growing malignancies derived from the notochord. Originally described in 1846 as an incidentally discovered small, myxoid exophytic lesion of the dorsum sella, uncertainty about its embryological origin persisted until the end of the twentieth century. At that time, a combination of clinical and laboratory evidence demonstrated that it was notochord-derived and not cartilaginous in origin, as had been previously argued. Treatment of chordomas has evolved over the 150 years since Virchow’s original description. Early descriptions of chordoma are draw from autopsy series, as surgical intervention was felt to be too high risk for the overwhelming majority of lesions. But with the advancement of surgical techniques, clinical evidence began to accumulate that supported surgical excision as the treatment of choice. Additionally, in the latter half of the twentieth century, high-dose radiation with stereotactic radiosurgery or charged particle therapy was seen as a means of improving local control in lesions where en bloc resection was infeasible. In this chapter, we review the discovery of chordomas origins and the evolution of treatment paradigms. We conclude with a description of the epidemiology of chordoma and clinical presentation.

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Abbreviations

BNCT:

Benign notochordal cell tumor

CGE:

Cobalt gray equivalent

Gy:

Gray

MGH:

Massachusetts General Hospital

NCI:

National Cancer Institute

SEER:

Surveillance, Epidemiology, and End Results

US:

United States

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Authors and Affiliations

  1. Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Zach Pennington, Andrew Schilling & Daniel M. Sciubba

  2. Department of Orthopaedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joseph H. Schwab

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    Daniel M. Sciubba

  2. Department of Orthopaedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joseph H. Schwab

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Pennington, Z., Schilling, A., Schwab, J.H., Sciubba, D.M. (2021). Historical Overview, Demographics, and Clinical Presentation of Spinal Chordoma. In: Sciubba, D.M., Schwab, J.H. (eds) Chordoma of the Spine. Springer, Cham. https://doi.org/10.1007/978-3-030-76201-8_3

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