The purpose of this study was to evaluate the
long-term outcome of patients with a sacral chordoma and the surgical
management of locally recurrent disease. Between October 1990 and August 2013 we operated on 54 consecutive
patients with a sacral chordoma. There were 34 men and 20 women
with a mean age of 60 years (25 to 86). The mean maximum diameter
of the tumour was 9.3 cm (3 to 20). The mean follow-up was 7.8 years (2 months to 23.4 years). The
disease-specific survival was 82% at five years, 57% at ten years
and 45% at 15 years. The local recurrence-free survival was 49%
at five years, 37% at ten years and 20% at 15 years. Local recurrence
occurred in 30 patients (56%) at a mean of 3.8 years (3 months to
13 years) post-operatively. Survival after the treatment of recurrence was 89% at two years,
56% at five years and 19% at ten years. Of nine patients who had
complete resection of a recurrence, one died after 72 months and
eight remain disease-free. Incomplete resection of recurrent disease
resulted in a survival of 54% at two years and 36% at five years. For 12 patients with a local recurrence who were treated palliatively,
survival was 81% at two years and 31% at five years. A wide margin of resection gave the best chance of long-term
survival and complete resection of local recurrence the best chance
of control of disease. Cite this article:
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
