Dermatofibrosarcoma protuberans (DFSP) – advances in diagnostics and therapy Review article
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Abstract
Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma with a superficial growth. It has an estimated incidence of 4 cases per one million persons per year. DFSP is preferentially located on the trunk. It can appear at any age, although it is much more frequent in individuals aged between 20 and 50 years. Its growth is slow and indolent and it can last for years. With time it can accelerate and characteristic protuberant masses appear, from which the disease took its name. Distant metastases are rare, more common in the fibrosarcomatous type (FS-DFSP), with incidence of about 10% of all DFSP, and it is characterized by more aggressive behavior and poorer prognosis. In more than 95% of DFSP cases a characteristic genetic disturbance was demonstrated, which is responsible for its carcinogenesis. Translocation between chromosomes 17 and 22 results in excessive activation of PDGFRβ and in increasing of the tumor cells proliferation. Excision with wide margins is the primary treatment option for DFSP. Though improvement of surgical modalities, including microsurgery, local recurrence rates are still high. Outcomes are better with the use of postoperative radiotherapy. Using in therapy kinase inhibitor imatinib was a breakthrough in treatment of patients with locally advanced or metastatic disease. Its activity is based on inhibition of PDGFRβ activation, which is responsible for development of this tumor type.
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Copyright: © Medical Education sp. z o.o. This is an Open Access article distributed under the terms of the Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). License (https://creativecommons.org/licenses/by-nc/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
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