Myxofibrosarcomas are soft tissue malignant tumours that are grossly and microscopically nodular and mucoid in appearance. They tend to occupy subcutaneous sites, that characteristically have a large vascular network with rapid circulation (Kindblom et al., 1977, Fletcher et al., 2002). The differential diagnosis includes other soft tissue sarcomas such as leiomyosarcoma and liposarcoma (Mutter et al., 2011).
These tumours tend to occur in the elderly population, commonly affecting the extremities (lower > upper), and are typically prevalent in male patients aged sixty to eighty years (Fletcher et al., 2002, Sanfilippo et al., 2011). Their presentation is often that of a painless slowly growing mass.
The risk of distant metastases is generally lower than for other soft tissue sarcomas. Metastatic sites include lung, bone and lymph nodes, with histological grade being the main risk factor for metastasis (Mentzel, 2011, Sanfilippo et al., 2011).
Myxofibrosarcomas tend to grow by tracking along fascial planes, which can be manifested on MRI by a high signal “tail” which contains tumour (Kaya et al., 2008). Even in the absence of this appearance, these tumours tend to grow in an infiltrative fashion. The risk of local recurrence is consequently high compared to other sarcomas (31% in one series (Haglund et al., 2010)). When it occurs, local recurrence is associated with more chromosomal aberrations and a higher histological grade, so increasing the likelihood of invading distant sites; overall survival at five years has been reported as being 60-77% (Fletcher et al., 2002, Willems et al., 2006, Sanfilippo et al., 2011).
Management should be multidisciplinary, with a surgical approach emphasising negative margins. As with other soft tissue sarcomas, adjuvant radiotherapy may be useful (Mutter et al., 2011, Sanfilippo et al., 2011).
Christopher Ghazala, Craig Gerrand
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