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Chordoma

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Tumors and Tumor-Like Lesions of Bone

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Abstract

Chordoma is a rare malignant tumor with notochordal differentiation. It is most common in 40–70 years of age male patients, 50 % of cases involving sacrococcygeal bones, 20–40 % skull base, and 10–30 % the mobile spine. Extra-axial cases have been reported. Radiologically, it is a destructive expanding lesion in the midline of the affected segment. Histologically, it has a lobular arrangement of cells with eosinophilic cytoplasm and intracytoplasmic vacuoles showing “physaliphorous” appearance. It is immunohistochemically positive for epithelial markers, brachyury, S100, and vimentin. It must be differentiated mainly from chondrosarcoma, metastatic carcinoma, and myxopapillary ependymoma. It has a slow infiltrative growth with frequent recurrences. There is a poor prognosis for dedifferentiated chordoma. Surgery is the mainstay of treatment.

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Authors and Affiliations

  1. Laboratory of Anatomic Pathology, Kyoto University Hospital, Kyoto, Japan

    Yasuaki Nakashima MD, PhD

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  1. Yasuaki Nakashima MD, PhD
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Correspondence to Yasuaki Nakashima MD, PhD .

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Editors and Affiliations

  1. Laboratory of Orthopaedic Pathology, Buenos Aires, Argentina

    Eduardo Santini-Araujo

  2. Laboratory of Orthopaedic Pathology, Buenos Aires, Argentina

    Ricardo K. Kalil

  3. Villa Erbosa Hospital, University of Bologna, Bologna, Italy

    Franco Bertoni

  4. Department of Pathology, Kyung Hee University Hospital, Seoul, Korea, Republic of (South Korea)

    Yong-Koo Park

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© 2015 Springer-Verlag London

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Nakashima, Y. (2015). Chordoma. In: Santini-Araujo, E., Kalil, R., Bertoni, F., Park, YK. (eds) Tumors and Tumor-Like Lesions of Bone. Springer, London. https://doi.org/10.1007/978-1-4471-6578-1_39

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  • DOI: https://doi.org/10.1007/978-1-4471-6578-1_39

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-6577-4

  • Online ISBN: 978-1-4471-6578-1

  • eBook Packages: MedicineMedicine (R0)

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