Dermatofibrosarcoma Protuberans of the Forehead: Case Report of a Rare Entity and Review of Literature
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma originating from fibroblasts in the dermal connective tissue, comprising approximately 1% of all soft tissue sarcomas. While most cases involve the trunk and extremities, only 10%–15% occur in the cephalic region, representing less...
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| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/crom/9991548 |
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| Summary: | Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma originating from fibroblasts in the dermal connective tissue, comprising approximately 1% of all soft tissue sarcomas. While most cases involve the trunk and extremities, only 10%–15% occur in the cephalic region, representing less than 1% of all head and neck neoplasms. DFSP is notable for its high propensity for local recurrence following surgical excision and its low metastatic potential. We report a case of recurrent DFSP of the forehead extending to the anterior wall of the left frontal sinus, without brain involvement, in a 33-year-old male with a history of three prior wide local excisions. A multidisciplinary cancer team recommended systemic imatinib therapy. This case highlights the challenges of managing DFSP in an uncommon location, underscoring the importance of a multidisciplinary approach in addressing recurrent and complex presentations. |
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| ISSN: | 2090-6714 |