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Chordoma and Chondrosarcoma

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Atlas of Sellar and Parasellar Lesions

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Abstract

Chordomas are locally aggressive tumors originating from notochord remnants along the neural axis, especially at the rostral and caudal extents (clivus and sacrum).

Typically, these invasive, expansile lesions present secondary to mass effect on surrounding structures.

The median age at presentation is 38–46 years; the age for chondrosarcoma may be slightly higher.

Overall, chordomas occur more frequently in men, but intracranial chordomas demonstrate an even predilection for both sexes.

Chordomas occur most frequently in the sacral region (50 %), followed by the clivus (35 %) and additional spinal regions (10–15 %).

Less commonly, they present primarily as intrasellar, retrosellar, nasopharyngeal, or foramen magnum region lesions.

Chordomas comprise 0.5–2 % of sellar lesions in major transsphenoidal series.

Diplopia caused by abducens nerve paresis is the most common presenting sign of a clival chordoma. The abducens nerve enters Dorello’s canal in the clivus and ascends until it enters the cavernous sinus, thus making it susceptible to injury by the tumor or during surgical resection of chordomas.

Other commonly associated symptoms and signs are headache, visual loss, hypopituitarism, weakness, and disequilibrium.

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Authors and Affiliations

  1. Department of Neurological Surgery, Keck School of Medicine, University of Southern California, 1200 N State Street, #3300, Los Angeles, CA, 90033, USA

    Gabriel Zada MD, MS

  2. Department of Pathology (Neuropathology), University of Virginia School of Medicine, 1215 Lee Street, Room 3060–HEP, Charlottesville, VA, 22908, USA

    M. Beatriz S. Lopes MD, PhD

  3. Department of Radiology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Srinivasan Mukundan Jr. PhD, MD

  4. Department of Neurosurgery, Brigham and Women’s Hospital, 15 Francis Street, Boston, MA, 02115, USA

    Edward Laws Jr. MD

Authors
  1. Gabriel Zada MD, MS
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  2. M. Beatriz S. Lopes MD, PhD
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  3. Srinivasan Mukundan Jr. PhD, MD
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  4. Edward Laws Jr. MD
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Corresponding author

Correspondence to Gabriel Zada MD, MS .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, University of Southern California, LOS ANGELES, California, USA

    Gabriel Zada

  2. Department of Pathology, University of Virginia, CHARLOTTESVILLE, Virginia, USA

    M. Beatriz S. Lopes

  3. Department of Radiology, Brigham and Women's Hospital, BOSTON, Massachusetts, USA

    Srinivasan Mukundan Jr.

  4. Department of Neurosurgery, Brigham and Women’s Hospital, BOSTON, Massachusetts, USA

    Edward R. Laws Jr.

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Zada, G., Lopes, M.B.S., Mukundan, S., Laws, E. (2016). Chordoma and Chondrosarcoma. In: Zada, G., Lopes, M., Mukundan Jr., S., Laws Jr., E. (eds) Atlas of Sellar and Parasellar Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-22855-6_31

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  • DOI: https://doi.org/10.1007/978-3-319-22855-6_31

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22854-9

  • Online ISBN: 978-3-319-22855-6

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