Abstract

BARRERA, Juan Camilo; ACOSTA, Álvaro Enrique  and  TRUJILLO, Laura. Dermatofibrosarcoma protuberans. rev.colomb.cancerol. [online]. 2019, vol.23, n.3, pp.99-109.  Epub Oct 26, 2019. ISSN 0123-9015.  https://doi.org/10.35509/01239015.38.

Dermatofibrosarcoma protuberans (DFSP) is a low incidence and slow growing cutaneous sarcoma that mainly affects individuals between 20 and 39 years old and is more frequent in black race. The most common anatomical site is the trunk, followed by the upper extremities. The etiology is unclear, but the most cases present genetic rearrangements involving the COL1A1 and PDGFB genes. The risk of metastasis is low, but it tends to local recurrence, especially when section margins were insufficient, in deep tumors or those with fibrosarcomatous change. The first line of treatment for local disease is surgery; wide local resection or Mohs micrographic surgery (MMS). The latter technique has shown consistently low recurrence rate. Radiotherapy has been used for unresectable cases or as adjuvant therapy. Imatinib is useful for metastatic disease.

Keywords : Dermatofibrosarcoma; Therapy; Surgery; Mohs surgery; Pathology.

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