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Introduction: Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive cutaneous soft tissue neoplasm, which appears more frequently in adults aged 30 years, being more common in blacks, with no significant difference in frequency between genders. The site where DFSP most commonly appears is on the trunk and proximal extremities, and should be suspected in any patient with a history of a firm, slow-growing cutaneous nodule. Dermoscopy findings may help in the suspicion of the disease, but the definitive diagnosis, which is usually late, requires an incisional biopsy. For a localized DFSP, the preferred initial treatment is radical resection with pathologically negative margins. As metastases to lymph nodes are very rare, prophylactic lymphadenectomy is not performed, it is only necessary when there is dissemination to the regional lymph node chain. Most recurrences are most evident within three years and follow-up should be done ad aeternum. Objective: To report a case of an unusual tumor of the DFSP type to provide discussion of the proposed treatment and manage future cases. Methods: Data for this case report were collected from medical records, test results and photographic records of meetings with the patient. Result: Male patient, white, 51 years old, sought care in January 2022 due to the appearance, at the age of 30, of a lesion in the thoracic region, with progressive growth. An incisional pectoral biopsy was performed with a histological and immunohistochemical findings showing DFSP, and the tumor was then resected, with free margins, and oral antibiotic therapy was indicated. Healing was by secondary intention, without the need for grafting. Conclusion: The established treatment had a curative purpose, with a radical excision with wide margins and negative for neoplasia, without the need for additional surgery. And due to the possibility of recurrence, the patient will maintain outpatient follow-up annually.
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