Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Chordoma, Mediastinal

  • Giovanni Falconieri
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-66796-6_11

Synonyms

Chordoid chordoma

Definition

Chordoma is a slowly growing malignant tumor of uncertain histogenesis thought to be derived from the embryonic notochord. It has a predilection for the cervical and lumbosacral spine. Although rarely, chordoma has been described to occur in the mediastinum as discrete masses with no apparent relationship with the adjacent vertebral bones (Ahrendt and Wesselhoeft 1992; Suster and Moran 1995). Presenting symptoms are related to local compression or invasion, mostly difficulty swallowing, chest pain, and shortness of breath.

Clinical Features

  • Incidence

    Not applicable due to the limited number of reported cases.

  • Age

    Most published cases have been seen in late adulthood. Exceptional cases are seen in pediatric subjects.

  • Sex

    No sex predilection.

  • Site

    Mediastinal chordomas arise in the posterior sector of the mediastinum.

  • Treatment

    Complete surgical excision is the treatment of choice for resectable tumor. Postoperative radiotherapy has been administered...

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References and Further Reading

  1. Ahrendt, M. N., & Wesselhoeft, C. W. (1992). Chordoma presenting as a posterior mediastinal mass in a pediatric patient [Case Reports Review]. Journal of Pediatric Surgery, 27(12), 1515–1518.CrossRefPubMedGoogle Scholar
  2. Suster, S., & Moran, C. A. (1995). Chordomas of the mediastinum: Clinicopathologic, immunohistochemical, and ultrastructural study of six cases presenting as posterior mediastinal masses [Case Reports]. Human Pathology, 26(12), 1354–1362.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pathology, School of MedicineUniversity of TriesteTriesteItaly