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Journal of Bone and Joint Surgery - British Volume, Vol 90-B, Issue 5, 652-656.
doi: 10.1302/0301-620X.90B5.20365  
Copyright © 2008 by British Editorial Society of Bone and Joint Surgery
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Dedifferentiated chordoma

A REPORT OF FOUR CASES ARISING ‘DE NOVO’

S. A. Hanna, MRCS, Research Registrar1; R. Tirabosco, MD, Consultant Histopathologist2; A. Amin, MRCS, Specialist Registrar1; R. C. Pollock, FRCS, Consultant Orthopaedic Surgeon1; J. A. Skinner, FRCS, Consultant Orthopaedic Surgeon1; S. R. Cannon, MCh(Orth), FRCS, Consultant Orthopaedic Surgeon1; A. Saifuddin, MRCP, FRCR, Consultant Radiologist3; and T. W. R. Briggs, MCh(Orth), FRCS, Consultant Orthopaedic Surgeon1

1 Department of Orthopaedic Oncology
2 Department of Histology
3 Department of Radiology Royal National Orthopaedic Hospital, Stanmore, Middlesex HA7 4LP, UK.

Correspondence should be sent to Mr S. A. Hanna; e-mail: sammyhanna{at}hotmail.com

Dedifferentiated chordoma is a rare and aggressive variant of the conventional tumour in which an area undergoes transformation to a high-grade lesion, typically fibrous histiocytoma, fibrosarcoma, and rarely, osteosarcoma or rhabdomyosarcoma. The dedifferentiated component dictates overall survival, with smaller areas of dedifferentiation carrying a more favourable prognosis. Although it is more commonly diagnosed in recurrences and following radiotherapy, there have been a few reports of spontaneous development. We describe four such cases, which were diagnosed de novo following primary excision, and discuss the associated clinical and radiological features.






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Hip, Knee, Trauma, Upper limb, Foot & Ankle, Paediatrics, Oncology, Spine, Arthroplasty, General