Abstract
Fibrosarcoma can occur at all ages (Fig. 12.1) and in all sites (Fig. 12.2). Before the era of immunohistochemistry, fibrosarcoma was a very fashionable diagnosis and represented one of the most common types of soft tissue sarcoma. With the development of immunohistochemical and molecular techniques, it is now rare for a sarcoma to be termed as fibrosarcoma, which by its name implies fibroblasts as the cell of origin. With increasing sophistication in diagnosis, more and more subtypes of fibroblastic sarcomas are now appreciated, all relatively rare tumors, but some show distinct molecular aberrations. While surgery for essentially all and radiation for some patients remain the standard of care for primary therapy for any of these soft tissue sarcomas, there has been less experience with each of these subtypes of tumors with respect to chemotherapy than with more common diagnoses. Beyond dermatofibrosarcoma protuberans, recommendations presented here are provisional and should be considered a starting point for prospective and hopefully multicenter clinical trials including patients with these diagnoses.
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Brennan, M.F., Antonescu, C.R., Alektiar, K.M., Maki, R.G. (2016). Fibrosarcoma and Its Variants. In: Management of Soft Tissue Sarcoma. Springer, Cham. https://doi.org/10.1007/978-3-319-41906-0_12
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DOI: https://doi.org/10.1007/978-3-319-41906-0_12
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