Document Type

Article

Publication Date

11-24-2023

Comments

This article is the author’s final published version in the Journal of Neurological Surgery Reports, Volume 84, Issue 4, 2023, Pages e146-e155.

The published version is available at https://doi.org/10.1055/s-0043-1777073. Copyright © 2023. The Author(s).

Publication made possible in part by support from the Jefferson Open Access Fund

Abstract

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.

Creative Commons License

Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 License.

PubMed ID

38026145

Language

English

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