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Clinical Dermatology

A case of oral dermatofibrosarcoma protuberans relapsing in the skin

Case-based review, 26 - 28
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Abstract
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Dermatofibrosarcoma protuberans comprises roughly 0.1% of all malignant tumors and approximately 1 to 2 percent of all soft tissue sarcomas. Slowly growing and locally aggressive, dermatofibrosarcoma protuberans can reach an enormous size, underlying in fascia and musculoskeletal structures including bone and also becoming superficial. It is characterized by high rates of local recurrence and low risk of metastasis. Dermatofibrosarcoma protuberans may involve the head and neck area in 10-15% of cases, but it is rarely described in the oral mucosa.
Here, we describe a case of oral dermatofibrosarcoma protuberans relapsing in the skin after 18 years. The lineage of the cell type comprising dermatofibrosarcoma protuberans remains a matter of controversy, but this case is consistent with the opinion that the progenitor cell is not only present in the skin but also in the submucosal tissue of the oral cavity.

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