Korean J Dermatol.  2022 Jan;60(1):53-56.

Dermatofibrosarcoma Protuberans with Diversely Atypical Clinical Presentation: Three Case Reports

• Lee SH
• Lee EH
• Han MH
• Lee SJ

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare, slow-growing dermal spindle cell tumor characterized by high local recurrence rates and a low risk of metastasis. It starts as an erythematous indurated papule or plaque where firm nodules subsequently arise, forming typical ‘protuberant’ morphology. However, atypical DFSP presentations, which are difficult to diagnose clinically, have been reported. In this study, we describe three patients diagnosed with diversely atypical DFSP, which were pigmented, hemangioma-like, and cystic DFSP variants. Among them, two patients were misdiagnosed with other diseases, resulting in delayed treatment, with much larger resections needed to achieve local control or avoid recurrence. Therefore, dermatologists should pay attention to these diverse clinical variants of DFSP.

Keyword

Atypical morphology; Dermatofibrosarcoma protuberance; Diagnosis