A Case of Highly Recurrent DFSP: Treatment Dilemmas and Considerations

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue tumor affecting the dermis and subcutaneous tissues, with potential involvement of muscle and fascia. This case report describes a 68-year-old Caucasian male with a history of recurrent DFSP on the left cheek, initially excised 36 years ago, with multiple recurrences despite wide local excisions (WLEs), eventually requiring left orbital enucleation, presenting to the clinic with a 10-year history of a slow-growing lesion on the left temporal scalp. Examination revealed a 2 cm flesh-colored, firm nodule, which biopsy confirmed as DFSP. Despite two subsequent WLEs, positive margins persisted. The patient refused further surgical intervention and was referred for imatinib and radiation therapy, which he also declined. MRI revealed additional nodules near the left zygomatic arch and sternocleidomastoid. DFSP is diagnosed via biopsy, often confirmed with CD34 immunohistochemistry. Optimal treatment is Mohs micrographic surgery (MMS), but WLE is also used. The recurrence rate is high, especially in head and neck locations. This case underscores the necessity for multidisciplinary management and highlights the critical role of thorough physical and histopathologic examinations. Close clinical follow-up is essential due to the high recurrence risk within the first three years post-treatment. This report emphasizes the importance of early detection and comprehensive care strategies to manage DFSP effectively.