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This case report highlights the diagnostic challenges and therapeutic considerations when dermatofibrosarcoma protuberans (DFSP) present in atypical locations, such as the head and neck. A 46-year-old Hispanic male presented with a large, rapidly growing, protruding mass on the left medial forehead, initially diagnosed through ultrasound as a benign cyst. The lesion exhibited a significant increase in size over 5 months. Physical examination revealed a firm nodular mass without signs of inflammation or lymphadenopathy. The lesion was excised for biopsy, and histopathological analysis confirmed a diagnosis of DFSP, characterized by spindle cells arranged in a storiform pattern. Immunohistochemical staining revealed strong CD34 positivity, with negative markers for desmin, factor XIIIa, and SOX10. The tumor exhibited an infiltrative pattern, extending into the skeletal muscle. The patient was referred for wide local excision due to the complexity of achieving clear margins in a cosmetically sensitive area such as the forehead. DFSP most commonly occurs on the trunk and proximal extremities, with less frequent involvement of the head and neck. This case is notable for its atypical location on the forehead, making surgical excision particularly challenging due to the need for margin control while preserving facial esthetics. Initial misdiagnosis as a benign cyst highlights the diagnostic difficulty of DFSP in uncommon locations. The tumors rapid growth and deep infiltration into skeletal muscle are further complicated management, requiring careful surgical planning to prevent a recurrence. Long-term follow-up is essential due to the high recurrence rate of DFSP when margins are inadequate.
Raffa et al. (Thu,) studied this question.