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General Orthopaedics

Myxofibrosarcoma as a paradigm for the multidisciplinary management of soft tissue sarcoma

British Orthopaedic Association 2012 Annual Congress



Abstract

Background

Myxofibrosarcomas are malignant soft tissue tumours that often present as painless slowly growing masses in the extremities of older males. Locally infiltrative growth means risks of local recurrence is high. Management emphasises negative surgical margins and adjuvant therapy. The aim of this retrospective case series was to review our experience of this tumour, and make recommendations about a minimum resection margin and how best to utilise the expertise of the multidisciplinary team.

Methods

A computerised database identified patients with myxofibrosarcoma surgically treated in our centre between 1997 and 2011. Clinical records were reviewed. Margins were positive if tumour was at or within 1mm of the resection plane.

Results

43 patients (median age 68.6 years; 70% male) were identified. 42 underwent surgery: 26 (62%) by orthopaedics; 9 (21%) by plastics, and 7 (17%) jointly. The lower limb was the most common site (30/43, 70%). Mean tumour size was 5.9cm (range 1.5 to 20cm). 53% had grade III tumours. Of 39 tumours with available data, 21 were superficial fascially-based masses and 18 were deep.

23 (55%) had positive margins. Of these, 9 underwent re-excision, 5 of whom had adjuvant radiotherapy. 13 of the remaining 14 patients had adjuvant radiotherapy. Of the fascially-based tumours, there was microscopic spread beyond the macroscopic mass of between 3–25mm.

3 patients (7%) developed local recurrence at a mean of 25 months (14 to 30 months). 12 (29%) had metastases at a mean of 19 months (range 7 to 48 months).

Conclusion

Myxofibrosarcoma poses a number of challenges for sarcoma teams; in particular, high positive margin rates, risks of further surgery and local failure. The infiltrative nature makes it suited to management by multidisciplinary teams. Microscopic tumour can present up to 2.5cm from the macroscopic mass for fascially-based tumours, and teams should plan appropriately.