Myxofibrosarcoma

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

References

Fletcher C, Unni K, Mertens F (2002) World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press.

Haglund K,  Raut CP,  Nascimento AF, Wang Q, George S, Baldini EH (2010)  Recurrence Patterns and Survival for Patients with Intermediate- and High-Grade Myxofibrosarcoma. International journal of radiation oncology, biology, physics 18 October 2010 (Article in Press DOI: 10.1016/j.ijrobp.2010.08.042)

Kindblom LG, Merck C, Svendsen P (1977) Myxofibrosarcoma: a pathologico-anatomical, microangiographic and angiographic correlative study of eight cases. Br J Radiol 50:876-887.

Kaya M, Wada T, Nagoya S, Sasaki M, Matsumura T (2008)  MRI and histological evaluation of the infiltrative growth pattern of myxofibrosarcoma. Skeletal Radiol (2008) 37: 1085.

Mentzel T (2011) Sarcomas of the skin in the elderly. Clinics in Dermatology 29:80-90.

Mutter RW, Singer S, Zhang Z, Brennan MF, Alektiar KM (2011) The enigma of myxofibrosarcoma of the extremity. Cancer.

Sanfilippo R, Miceli R, Grosso F, Fiore M, Puma E, Pennacchioli E, Barisella M, Sangalli C, Mariani L, Casali P, Gronchi A (2011) Myxofibrosarcoma: Prognostic Factors and Survival in a Series of Patients Treated at a Single Institution. Annals of Surgical Oncology 18:720-725.

Willems SM, Debiec-Rychter M, Szuhai K, Hogendoorn PCW, Sciot R (2006) Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model. Mod Pathol 19:407-416.

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