A Rare Orbital Pathology: A Large Orbital Dermatofibrosarcoma Protuberans

Turk J Ophthalmol. 2022 Dec 28;52(6):436-439. doi: 10.4274/tjo.galenos.2022.46383.


Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of the dermis. It is a malignant, locally aggressive, and infiltrative tumor with frequent recurrence. In this case, a 44-year-old woman presented with a 15-year history of a swelling in the medial canthus of the right eye that caused tearing. Imaging revealed a septated mass isodense to soft tissue that had eroded the medial wall of the orbit. Macroscopic examination showed an elastic, gray-brown, encapsulated, irregular mass measuring 45x35x22 mm. The surgical margins were positive, so adjuvant radiotherapy was started. The patient was followed for 2 years without recurrence. According to the literature, this mass is the largest orbital DFSP treated by globe-sparing primary resection.

Keywords: Dermatofibrosarcoma protuberans; eye tearing; medial canthus tumor; orbital radiotherapy; orbital tumor.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Dermatofibrosarcoma* / diagnosis
  • Dermatofibrosarcoma* / pathology
  • Dermatofibrosarcoma* / surgery
  • Female
  • Humans
  • Lacrimal Apparatus* / pathology
  • Orbit
  • Skin Neoplasms* / diagnosis