Dermatofibrosarcoma protuberans in a pre-adolescent child

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare skin sarcoma with a slow growth rate and less chance of metastasizing but it is associated with higher morbidity due to its aggressive nature of local infiltration and its recurrence nature. IMPORTANCE: When dealing with DFSP in children we could achieve low morbidity with appropriate surgical planning and approach even though aggressive wide local excision was performed. CASE REPORT: 12-year boy presented with a lump in his left arm which was initially clinically and ultrasonically diagnosed as lipoma. However, histological examination, coupled with CD34 immunohistochemistry, confirmed the diagnosis as DFSP. CLINICAL DISCUSSION: Modern microsurgical methods, such as Mohs micrographic surgery, are advocated as effective treatment options, their availability and feasibility may be limited in certain settings. Therefore, the classic approach of wide local excision remains the treatment of choice in the majority of cases, with radiotherapy recommended for recurrent disease. Proper patient education and regular surveillance will help identify recurrence in time which aids us to provide timely intervention and optimize outcomes for patients with dermatofibrosarcoma protuberans. CONCLUSION: Accurate diagnosis using histology coupled with immunohistochemistry, proper surgical technique and regular follow up are the three pillars of managing DFSP.