Reporte de caso clínico: paciente con dermatofibrosarcoma protuberans en el municipio de Armenia, Quindío

Dermatofibrosarcoma is a rare, locally aggressive cutaneous tumor that is generated due to a genetic translocation that results in the fusion of the PDFGB (Beta-polypeptide-derived growth factor of platelets) genes with the COL1A1 gene (Collagen Type 1A1) generating a continuous autocrine activation of said fusion resulting in a constant and uncontrolled growth of tumor cells. It accounts for between 1 and 6% of all soft tissue sarcomas and 85% of these are of low grade, originating mainly in the fourth decade of life, although they have been occasionally reported in elderly and congenital, similar to men and women with a slight predominance in the male gender. The diagnosis usually is made by clinical diagnosis, history of the patient, evolution and characteristics of the lesion, however, due to the risk of deep tissue invasion or suspicion of another type of tumor lesion, it should always be made definitive through biopsy and histopathology. Initial management should always be through surgical excision, with wide margins, if necessary with MOHS technique, and in rare cases radiotherapy may be necessary as a post-surgical adjuvant treatment. In the present case study, a dermatofibrosarcoma was diagnosed in a 60-year-old male patient who presented a lesion of approximately 20 years, which was recurrent and was managed through extensive surgical excision with subsequent autologous skin transplantation for defect correction; a satisfactory resection of the lesion, and a favorable postoperative period was obtained. It should be taken into account that the prognosis is favorable in most cases, however there are risk factors of poor prognosis, such as history of recurrence, male sex and location in the extremities, all of them present in this case. ACRONYMS: PDFGB (Platelet-derived beta-polypeptide growth factor). COL1A1 (Collagen type 1A1).

• Autores: Flórez, Carlos Javier; Gonzalez Walles, Jonathan; Jaramillo Acosta, Manuel Alejandro; Arango, Carlos Eduardo
• Tipo de recurso: Article of investigation
• Fecha de publicación: 2017
• Institución: Universidad del Quindío
• Repositorio: Repositorio Universidad del Quindío
• Idioma: spa
• OAI Identifier: oai:bdigital.uniquindio.edu.co:001/6581
• Acceso en línea: https://bdigital.uniquindio.edu.co/handle/001/6581
• Palabra clave: Dermatofibrosarcoma protuberans; Fibrohistiocito; Dermatofibrosarcoma protuberans,; Fibrohistiocyti
• Rights: openAccess
• License: https://creativecommons.org/licenses/by-nc-nd/4.0/