Published online Oct 26, 2025. doi: 10.12998/wjcc.v13.i30.111020
Revised: July 18, 2025
Accepted: August 13, 2025
Published online: October 26, 2025
Processing time: 112 Days and 22.6 Hours
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, locally aggressive cutaneous sarcoma. DFSP in the periocular region is exceedingly rare, leading to diagnostic and surgical challenges due to anatomical constraints in the periocular region. Precise diagnosis is essential to guide appropriate surgical management and prevent recurrence.
A 32-year-old female presented with a recurrent tumor in the medial canthus, previously diagnosed as a solitary fibrous tumor in an outside institution. After complete radiological and systemic workup, she was scheduled for a wide local excision followed by reconstruction after getting tumor clear margins on frozen section. Histopathology confirmed DFSP, characterized by storiform spindle cell proliferation, diffuse cluster of differentiation 34 positivity, and signal transducer and activator of transcription 6 negativity.
This case highlights the challenges in the diagnostic and surgical management of DFSP in periocular tumors. Comprehensive surgical excision with appropriate reconstruction is critical for achieving oncological control while preserving aes
Core Tip: Periocular dermatofibrosarcoma protuberans (DFSP) of the lacrimal sac is extremely rare, often mimicking benign or inflammatory lesions, leading to delayed diagnosis. High clinical suspicion is warranted for slowly enlarging, firm, painless medial canthal masses. Histopathology with cluster of differentiation 34 immunopositivity confirms the diagnosis. Complete surgical excision with histologically clear margins is crucial to prevent recurrence, with consideration for Mohs micrographic surgery in select cases. Long-term follow-up is essential due to the locally aggressive nature of DFSP and potential for late recurrence. Early recognition and meticulous margin control are key to optimal functional and cosmetic outcomes.