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Dermatofibrosarcoma protuberans

Neoplasms

Dermatofibrosarcoma protuberans, often abbreviated DFSP, is a rare, locally aggressive malignant cutaneous soft-tissue sarcoma. It develops in the connective tissue cells in the middle layer of the skin, the dermis. Estimates of its overall occurrence in the United States range from 0.8 to 4.5 cases per million persons per year. In the United States it accounts for between 1 and 6 percent of all soft-tissue sarcomas and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma among cutaneous soft-tissue sarcomas.

Facts
Classification
Body System
Integumentary System 1
ICD-10 Chapter
II: Neoplasms 1
Communicable or Non-communicable
Non-Communicable 1
First Described
1890 1
Sources
1. Dermatofibrosarcoma protuberans (Wikipedia)
  • Lead, first sentence
    is a rare locally aggressive malignant cutaneous soft-tissue sarcoma
  • Lead, second sentence
    DFSP develops in the connective tissue cells in the middle layer of the skin (dermis)
  • Lead: disease category
    Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma.
  • History section
    Taylor, in 1890, first identified DFSP as a keloid sarcoma.
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