Dedifferentiated chordoma: a report of four cases arising 'de novo'.
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.
Hanna
S A
SA
Department of Orthopaedic Oncology, Royal National Orthopaedic Hospital, Stanmore, Middlesex HA74LP, UK. sammyhanna@hotmail.com
Tirabosco
R
R
Amin
A
A
Pollock
R C
RC
Skinner
J A
JA
Cannon
S R
SR
Saifuddin
A
A
Briggs
T W R
TW
D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000473:pathology* / Q000628:therapy; D002817:Chordoma / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged