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Updated: May 13, 2026
06:15
Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia
Published on: August 9, 2024
Primary dermatofibrosarcoma protuberans of orbit--a rare entity.
Tashnin Rahman1, Kasturi Bhattacharjee, Jagannath Dev Sarma
1Dr. B. Borooah Cancer Institute, Regional Cancer Centre, Department of Head and Neck Oncology, Assam, Guwahati, India. drtashnin@yahoo.in
Orbit (Amsterdam, Netherlands)
|March 2, 2013
Summary
A rare case of primary orbital Dermatofibrosarcoma Protuberance (DFSP) was reported in a 70-year-old woman. This unique orbital tumor required surgical removal via orbital exenteration.
Area of Science:
- Oncology
- Ophthalmology
- Dermatology
Background:
- Dermatofibrosarcoma Protuberance (DFSP) is a rare, locally aggressive skin neoplasm with a high recurrence rate.
- Orbital involvement by DFSP is uncommon, typically occurring secondary to metastasis or local invasion.
- Primary DFSP originating within the orbit is exceptionally rare.
Observation:
- This report details a unique case of primary orbital DFSP in a 70-year-old female patient.
- The patient underwent orbital exenteration as the primary treatment modality.
- Diagnostic evaluation included histopathology and immunohistochemistry.
Findings:
- Histopathology examination revealed characteristic spindle cells arranged in a storiform pattern.
- Immunohistochemistry confirmed the diagnosis with CD34 positivity and S100 negativity.
Implications:
- This case highlights the importance of considering primary orbital DFSP as a distinct entity.
- Accurate diagnosis through HPE and IHC is crucial for appropriate management of orbital tumors.
- Understanding the unique presentation of primary orbital DFSP can aid in early detection and treatment strategies.
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