Dermatofibrosarcoma protuberans (DFSP) is an uncommon skin cancer with a sluggish growth rate. As long as the cancer is treated before it spreads, the prognosis for those with DFSP is excellent.
A rare kind of cancer known as dermatofibrosarcoma protuberans (DFSP) starts in the dermis, the middle layer of the skin.
Although it can affect individuals of various ages, DFSP most frequently affects adults in the 20–50 age range. Rarely does the tumor spread to other parts of the body and usually grows slowly.
But over time, it may spread into the surrounding muscle, fat, or even bone tissue, which would make treatment challenging and negatively impact your quality of life.
Learn about DFSP, its symptoms, diagnosis, available treatments, and the prognosis for those who have it by continuing to read.
What are the symptoms of dermatofibrosarcoma protuberans?
DFSP typically begins as a little, rigid patch of skin that is hard or rubbery to the touch. It may resemble a dermatofibroma, a common, benign skin growth.
Because the intermediate layer of the skin presses against the upper layer, the patch may develop and eventually become a raised lump known as a protuberance.
Typical signs of DFSP include:
- a little cutaneous lesion that develops slowly
- a hard, elevated nodule that may be brown, red, or purple in color.
- discomfort or soreness in the vicinity of the injury
- bleeding from the wounded area, however infrequently
People with DFSP may wait years to seek medical attention, which can postpone diagnosis and therapy because the condition advances slowly.
How do doctors diagnose dermatofibrosarcoma protuberans?
There are usually multiple processes involved in diagnosing DFSP.
First, a physical examination and medical history are taken by a doctor. They’ll probably advise a skin biopsy to verify the diagnosis. A tiny sample of the afflicted skin tissue will be removed during a biopsy, and it will be examined under a microscope.
Different types of biopsies may be used by doctors, such as:
- Core needle biopsy: A medium-sized needle is used by doctors to remove a column of tissue.
- Excisional biopsy: a surgical technique when a lump is excised in its entirety for analysis by the surgeon.
Doctors may advise imaging tests like MRIs or CT scans in addition to biopsies to assess the tumor’s extent and look for any potential metastases to adjacent tissues.
What is the treatment for dermatofibrosarcoma protuberans?
Surgery is the primary treatment for DFSP. The intention is to remove the tumor in its entirety as well as some of the surrounding healthy tissue. If not, the tumor can regrow or reoccur. The two primary surgical methods employed by physicians are:
- Wide local excision: A surgeon excises the tumor and surrounding healthy tissue with a knife.
- Mohs micrographic surgery: Until no tumor cells are left, a surgeon removes the malignant cells layer by layer while closely analyzing each layer under a microscope. Although it takes a long time, this method helps to maintain the greatest amount of good tissue.
A margin of two to four centimeters (cm), or the space of healthy tissue surrounding the tumor, is advised by the National Comprehensive Cancer Network (NCCN).
In some circumstances, more therapies could be required, like:
- Radiation therapy: If the tumor is in a place that makes surgery challenging or if some cancer cells survive surgery, a doctor might advise radiation therapy.
- Targeted therapy: Imatinib (Gleevec), one example of a targeted therapy, targets particular chemicals that contribute to the proliferation of cancer cells. When surgery is not an option or for advanced DFSP, doctors may suggest this.
After treatment, follow-up care is essential to spotting any early recurrence signals and acting upon them. Your medical staff will probably teach you how to perform self-examinations and arrange for routine checkups.
What is the outlook for people with dermatofibrosarcoma protuberans?
For those who are diagnosed with DFSP early and have it surgically removed, the prognosis is usually favorable. Rarely does DFSP expand to other bodily areas. For DFSP, the total 10-year survival rate is 99%.
Recurrence is typical, though. After a straightforward excision, the cancer returns in nearly half of DFSP patients. A larger excision margin greatly improves your prognosis, according to research.
The prognosis varies in situations of more advanced DFSP (spread to other tissues). Additional therapies like radiation or targeted therapy can be necessary for some patients.
After diagnosis, individuals with spread-related DFSP typically have a two-year life expectancy.
What causes dermatofibrosarcoma protuberans?
Although the precise etiology of DFSP is unknown, scientists think it may be related to genetic alterations in skin cells.
A translocation—the splitting and reattachment of a portion of one chromosome to another chromosome—between chromosomes 17 and 22 is one frequent genetic alteration linked to DFSP. A protein that stimulates the development of cancer cells is produced due to this alteration.
Who is at risk of dermatofibrosarcoma protuberans?
The following risk factors could make developing DFSP more likely:
- Age: Adults between the ages of 20 and 50 are the most common age range for DFSP diagnoses.
- Ethnicity: DFSP is more common in African Americans.
- Previous skin injuries: In the presence of a prior skin injury, such as a burn, surgery, or tattoo, DFSP may manifest.
Takeaway
A rare but curable form of skin cancer is called DFSP. For this condition to be effectively managed, early detection and timely treatment are essential. See a dermatologist as soon as you discover any strange changes or growths on your skin.
The primary course of treatment is surgical removal, with a typically favorable prognosis. It is crucial to have routine check-ups to make sure the cancer doesn’t return.
You can take charge of your health and well-being by being aware of your treatment options and having a thorough understanding of DFSP.