Definition
A malignant tumor usually composed of spindle and pleomorphic cells embedded in a myxoid stroma with curvilinear blood vessels.
Clinical Features
Incidence
Myxofibrosarcoma is a rare soft tissue sarcoma with an estimated incidence of <0.1/100.000/year (Stiller et al. 2013). Nevertheless, it is one of the most common sarcomas in adulthood.
Age
Myxofibrosarcoma affects adults, mostly elderly patients (peak of incidence in the seventh decade). However, cases occur in young adults, but almost never in children (Mentzel et al. 1996; Merck et al. 1983).
Sex
There is a slight male predominance (Mentzel et al. 1996; Merck et al. 1983).
Site
About two-thirds of myxofibrosarcomas develop within the dermis and subcutis while the remaining one-third in deep (subfascial) soft tissues. Most myxofibrosarcomas affect lower extremities (thigh, buttock, leg, and knee), followed by upper extremities (arm, shoulder, and forearm), trunk, and seldom...
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References and Further Reading
Angervall, L., Kindblom, L. G., & Merck, C. (1977). Myxofibrosarcoma: A study of 30 cases. Acta Pathologica Microbiologica Scandinavica Section A Pathology, 85(2), 127–140.
Colia, V., Fiore, M., Provenzano, S., Fumagalli, E., Bertulli, R., Morosi, C., … & Sanfilippo, R. (2017). Activity of anthracycline-and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma. Clinical Sarcoma Research, 7, 16.
Mentzel, T., Calonje, E., Wadden, C., Camplejohn, R. S., Beham, A., Smith, M. A., & Fletcher, C. D. (1996). Myxofibrosarcoma: Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. The American Journal of Surgical Pathology, 20, 391–405.
Merck, C., Angervall, L., Kindblom, L. G., & Oden, A. (1983). Myxofibrosarcoma. A malignant soft tissue tumour of fibroblastic-histiocytic origin. Acta Pathologica, Microbiologica et Immunologica Scandinavica, 81, 7–11.
Stiller, C. A., Trama, A., Serraino, D., Rossi, S., Navarro, C., Chirlaque, M. D., … & RARECARE Working Group. (2013). Descriptive epidemiology of sarcomas in Europe: Report from the RARECARE project. European Journal of Cancer, 49, 684–695.
Weiss, S. W., & Enzinger, F. M. (1977). Myxoid variant of malignant fibrous histiocytoma. Cancer, 39(4), 1672–1685.
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Cappellesso, R. (2020). Myxofibrosarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5434-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5434-1
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