Chordoma
For patient information, click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Notochordoma; chordocarcinoma; chordoepithelioma
Overview
Chordomas are uncommon malignant tumours that account for 1% of intracranial tumours and 4% of all primary bone tumours.
They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasise
Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within the tumour. In some patients, calcification and sequestered bone fragments are found as well. The variety of these components may explain the signal heterogeneity observed on MRI. Incomplete delineation of the tumour and microscopic distal extension of tumour cells may explain the frequency of recurrences. Physaliphorous cells are classically seen on microscopy
Metastatic spread of chordomas is observed in 7-14% of patients and includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement, predominantly from massive tumours. True malignant forms of chordomas occasionally have areas of typical chordoma and undifferentiated areas, most often suggestive of fibrosarcoma; the prognosis is poor.