is recommended. Chemotherapy DFSP was previously regarded and nonresponsive to standard chemotherapy. In 2006 the US FDA approved (imatinib mesylate) for the treatment of DFSP. As is true for all medicinal drugs that have a name that ends in "ib," imatinib is a small molecular pathway inhibitor; imatinib inhibits tyrosine kinase. It may be able to induce tumor regression in patients with recurrent DFSP, unresectable DFSP, or metastatic DFSP. There is clinical evidence that imatinib, which inhibits PDGF-receptors, may be effective for tumors positive for the t(17;22) translocation. It is suggested that imatinib may be a treatment for challenging, locally advanced disease and in the rare metastatic cases It was approved for use by adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP). Metastatic disease Distant hematogenous metastases are extremely rare. Metastases to regional lymph nodes are rarer and are most likely in patients who have had multiple local recurrences after inadequate surgical resection. Repeatedly recurring tumors have an increased risk for transformation into a more malignant form (DFSP-FS). The lungs are most frequently affected, but metastases to the brain, bone, and other soft tissues are reported. Studies DFSP is not extensively studied due to its rarity and low mortality. The majority of studies are small size case studies or meta-analysis. The most extensive research study to date was Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance. The lead researcher Jerad Gardner spoke at a Ted Talk in February 2020 on the topic. History Taylor, RW, in 1890. first identified DFSP as a keloid sarcoma. Later in 1924, Darier, J and Ferrand, identified it as a progressive recurrent dermatofibroma. In 1925 it was E Hoffmann who coined the term Dermatofibrosarcoma protuberans. Bednar tumor was first described by Bednar in 1957. ICD Coding ICD-0: 8832/3 - dermatofibrosarcoma protuberans, NOS ICD-0: 8833/3 - pigmented dermatofibrosarcoma protuberans ICD-0: 8834/1 - giant cell fibroblastoma Fibrosarcomatous dermatofibrosarcoma protuberans: no distinct coding identified Additional images See also List of cutaneous conditions References External links Dermatofibrosarcoma protuberans in NIH Genetic and Rare Diseases Information Center Dermal and subcutaneous growths Rare cancers Sarcoma