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Related Experiment Video

Updated: Mar 22, 2026

Intraoperative Ultrasound in Spinal Surgery

Intraoperative Ultrasound in Spinal Surgery

Published on: August 17, 2022

Extra-axial chordomas.

S Evans¹, Z Khan^1,2, L Jeys¹

¹Royal Orthopaedic Hospital NHS Foundation Trust , UK.

Annals of the Royal College of Surgeons of England

|April 19, 2016

Summary

This summary is machine-generated.

Extra-axial chordomas are rare malignant tumors originating outside the axial skeleton. Early diagnosis and specialist multidisciplinary team consultation are crucial for effective treatment of these uncommon bone cancers.

Keywords:

Chordoma Clinical course Extra-axial

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Area of Science:

Orthopaedic Oncology
Skeletal Tumors
Oncologic Pathology

Background:

Chordomas are slow-growing malignant tumors arising from notochordal remnants.
Histologically similar chordomas can occur outside the axial skeleton, but their clinical course is poorly documented.

Observation:

A 30-year review of an orthopaedic oncology database identified 131 chordoma cases.
Only three (2.3%) were extra-axial chordomas, located in the femur, ulna, and proximal fibula.
All three patients with extra-axial chordomas underwent surgical resection.

Findings:

Extra-axial chordomas represent a rare subset of chordoma, accounting for only 2.3% of cases in this series.
The rarity and diagnostic challenges of these tumors necessitate specialized evaluation.
Surgical resection was the primary treatment modality for the observed extra-axial chordoma cases.

Implications:

Accurate diagnosis of rare bone tumors like extra-axial chordomas requires expert input.
Multidisciplinary team (MDT) discussion is recommended for suspected cases to ensure appropriate diagnosis and treatment planning.
Specialized sarcoma MDTs can facilitate tailored management strategies for patients with challenging skeletal malignancies.