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Dermatofibrosarcoma protuberans
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a rare, slow-growing type of cancer that forms in the deep layers of the skin and has a high tendency to recur locally after surgical removal.

Overview & Symptoms

Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues.

In dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters in diameter, that is usually purplish, reddish, or flesh-colored. The tumor typically grows slowly and can become a raised nodule. Occasionally, the tumor begins as a flat or depressed patch of skin (plaque). Tumors are most commonly found on the torso and can also be found on the arms, legs, head, or neck. Affected individuals usually first show signs of this condition in their thirties, but the age at which a tumor appears varies widely.

In dermatofibrosarcoma protuberans, the tumor has a tendency to return after being removed. However, it does not often spread to other parts of the body (metastasize).

Variants

There are several variants of dermatofibrosarcoma protuberans in which different cell types are involved in the tumor:

  • Bednar tumors (often called pigmented dermatofibrosarcoma protuberans) contain dark-colored (pigmented) cells called melanin-containing dendritic cells.
  • Myxoid dermatofibrosarcoma protuberans tumors contain an abnormal type of connective tissue known as myxoid stroma.
  • Giant cell fibroblastoma (sometimes referred to as juvenile dermatofibrosarcoma protuberans because it typically affects children and adolescents) is characterized by giant cells in the tumor.
  • Fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) occurs when tumors have regions that look similar to fibrosarcoma, a more aggressive type of

Frequently Asked Questions

Dermatofibrosarcoma protuberans (DFSP) is characterized by long, thin cellular "tentacles" that extend deep into the surrounding tissue, often far beyond what is visible to the naked eye or even on standard imaging. This makes it extremely difficult to determine the exact boundaries of the tumor during surgery. Local recurrence means that the cancer returns in the same area where it was originally removed. Because of these microscopic extensions, standard excision often leaves behind cancer cells, leading to a high rate of regrowth unless a specialized surgical technique is used. How does Mohs micrographic surgery differ from standard excision, and why is it often the preferred treatment?::Mohs micrographic surgery is a specialized technique where the surgeon removes the tumor layer by layer, examining 100 percent of the surgical margins under a microscope immediately after each removal. This continues until no cancer cells are found at the edges. Unlike standard excision, which removes a fixed amount of tissue around the tumor and only samples a small percentage of the margins, Mohs surgery ensures that all visible and microscopic disease is removed. This approach significantly reduces the risk of the tumor coming back while preserving as much healthy tissue as possible. What are the signs that a previously treated DFSP might be recurring, and how often should follow-up appointments be scheduled?::Patients should monitor the treated area for any new firmness, thickening, or changes in skin color, such as redness or purple hues, even if the original scar looks healed. Because DFSP can recur many years after initial treatment, long-term surveillance is critical. Most guidelines recommend clinical examinations every six months for the first five years, and then annually thereafter for life. Patients should report any changes in the scar tissue immediately, as early detection of a recurrence allows for smaller, less complex surgeries.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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