| Summary: | Introduction: Dermatofibrosarcoma protuberans (DFSP) originated as keloid sarcoma, gaining its current designation in 1925. DFSP exhibits slow growth, categorizing it as a low- to intermediate-grade malignant sarcoma. Initially presenting as a small, firm, irregular skin nodule, it undergoes sudden, rapid growth, forming a prominent mass. While locally aggressive, distant metastasis is rare. DFSP affects mainly the torso then proximal extremities. Case Presentation: In this case study, we described a 57-year-old male individual who presented with a chest midline swelling that was progressing in size. A punch biopsy showed inconclusive results. Thus, a wide local excision was carried out along with sending the initial biopsy slides to Mayo Clinic for second opinion. A diagnosis of DFSP was confirmed, which is an uncommon and locally aggressive tumor affecting soft tissues. The primary histological diagnosis relies on immunohistochemical stains, enabling the distinction between DFSP and other fibrous tumors. Conclusion: Diagnosing DFSP is challenging due to its similarity to other skin lesions. A multidisciplinary approach is vital for accurate diagnosis and management.
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