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Orbitofacial dermatofibrosarcoma protuberans with intranasal extension.
Siti Nurhuda Sharudin1,2, Siow Wei Tan2, Nor Fadhilah Mohamad1
1a Department of Ophthalmology , University of Malaya Medical Centre , Kuala Lumpur , Malaysia.
Orbit (Amsterdam, Netherlands)
|October 24, 2017
Summary
This case report details a rare instance of recurrent dermatofibrosarcoma protuberans (DFSP) in the medial canthus and nasal region. Achieving clear surgical margins was challenging, highlighting the need for neoadjuvant therapy to prevent local relapse.
Area of Science:
- Oncology
- Dermatology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin cancer.
- DFSP typically presents on the trunk and extremities, rarely in the head and neck region.
- Accurate initial diagnosis is crucial for effective management.
Observation:
- A 25-year-old woman presented with recurrent painless swelling in the left medial canthus and an intranasal mass.
- Initial biopsy misdiagnosed the lesion as nodular fasciitis, leading to delayed diagnosis and treatment.
- The tumor extended from the dermis into the anterior orbit and nasal cavity.
Findings:
- Histopathology confirmed dermatofibrosarcoma protuberans (DFSP) after initial misdiagnosis.
- The tumor exhibited rapid local recurrence post-excision.
- Surgical resection with clear margins was challenging due to the tumor's extensive local invasion.
Implications:
- This case underscores the diagnostic challenges and aggressive nature of DFSP in unusual locations.
- Achieving negative surgical margins for DFSP can be difficult, necessitating multimodal treatment approaches.
- Neoadjuvant therapy may be beneficial in improving surgical outcomes and preventing local recurrence of DFSP.
