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Oncology

Dedifferentiated chordoma

A REPORT OF FOUR CASES ARISING ‘DE NOVO’



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Abstract

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.


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

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