CC BY-NC-ND 4.0 · J Neurol Surg Rep 2023; 84(04): e146-e155
DOI: 10.1055/s-0043-1777073
Case Report

Multifocal Ectopic Recurrence of a C2 Chordoma

1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
Allison Kayne
1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
Sarah Collopy
1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
Giyarpuram Prashant
1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
Patrick Kelly
1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
James J. Evans
1   Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
› Author Affiliations


Background Chordomas are histologically benign but locally aggressive tumors with a high propensity to recur. Our case highlights the importance of long-term vigilance in patients who have undergone chordoma resection.

Case Report We report the case of a 47-year-old man with a cervical chordoma who developed multiple musculoskeletal ectopic recurrences in the left supraclavicular region, the proximal right bicep, and the left submandibular region without recurrence in the primary tumor site. Primary tumor resection was achieved via a combination of surgery, adjuvant radiation therapy, and imatinib. All recurrences were successfully resected and confirmed via pathology to be ectopic chordoma.

Discussion Ectopic recurrence of cervical chordoma is rare and lung is the most common site of distant spread. Chordoma recurrence in skeletal muscle is particularly rare, with only 10 cases described in the literature. A plausible mechanism of distant metastatic disease in chordoma patients suggests that tumor cells escape the surgical tract via a combination of cytokine release, vasodilation, and microtrauma induced during resection.

Conclusion Cervical chordoma with ectopic recurrence in skeletal muscle has not been previously described in the literature. Skull base surgeons should be aware of the phenomenon of chordoma ectopic recurrence in the absence of local recurrence.

Publication History

Received: 24 January 2023

Accepted: 21 August 2023

Article published online:
24 November 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (

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