Dermatofibrosarcoma Protuberans (DFSP) Treatment
Specialized care for rare skin tumors—offering expert diagnosis and advanced surgery.
DFSP Is Rare but Treatable—Here’s What to Expect
Dermatofibrosarcoma Protuberans (DFSP) is a rare and locally aggressive type of skin cancer that originates from the connective tissue cells in the middle layer of the skin (the dermis). While DFSP is known for its potential to invade surrounding tissues, it has a low risk of spreading (metastasizing) to distant organs. The prognosis is excellent when DFSP is diagnosed early and treated appropriately.
At Phoenix Surgical Dermatology Group, our board-certified and fellowship-trained Mohs micrographic surgeon,
Dr. Ramin Fathi, offers specialized care for DFSP. With expertise in advanced surgical techniques, we aim to achieve the highest cure rates while preserving as much healthy tissue as possible.
What is Dermatofibrosarcoma Protuberans?
Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma that typically presents as a firm, slow-growing skin lesion. Although DFSP grows slowly, it is locally invasive and may extend into deeper layers of skin, fat, muscle, or even bone if left untreated.
This cancer can occur at any age but is most commonly diagnosed in adults between
20 and 50 years old. While it may appear anywhere on the body, the
trunk, arms, and legs are the most frequent sites of occurrence.
What Causes Dermatofibrosarcoma Protuberans?
Unlike many other skin cancers linked to sun exposure, DFSP is not associated with UV damage. Instead, DFSP results from a genetic mutation caused by the rearrangement of genetic material between chromosome 17 and chromosome 22. This abnormal translocation leads to continuous, uncontrolled cell growth.
Key Cause of DFSP:
- Random
chromosomal translocation (17 and 22)
- Resulting overproduction of
platelet-derived growth factor beta (PDGFB), which stimulates tumor cell growth
There are no well-established environmental or lifestyle risk factors for DFSP.
How Serious is Dermatofibrosarcoma Protuberans (DFSP)?
While metastasis is extremely rare in DFSP, the tumor is known for its high rate of local recurrence if not completely removed. The good news is that the 10-year survival rate for DFSP is over 99% when treated effectively.
The best treatment approach is
Mohs micrographic surgery, a technique that allows for the complete evaluation of cancer margins, offering the highest likelihood of cure with the least tissue removal.
Signs and Symptoms of DFSP
DFSP may be mistaken for other skin conditions because of its slow-growing nature and subtle appearance early on. Common symptoms and characteristics include:
- A
firm, raised bump or patch on the skin
- Red, pink, or purplish discoloration
- Lesion that
grows slowly over time
- Sometimes a
scar-like or dimpled appearance
- Commonly found on the
trunk, arms, shoulders, or legs
Because DFSP can resemble benign skin growths, it is essential to seek evaluation from a dermatologist for any suspicious, persistent lesion.
How Is DFSP Diagnosed?
Diagnosis of DFSP involves a combination of clinical examination and laboratory testing:
- Full skin examination by a board-certified dermatologist.
- Skin biopsy—removal of a tissue sample from the lesion, typically performed under local anesthesia.
- Microscopic analysis of the biopsy to confirm the diagnosis and identify the tumor type and characteristics.
- In certain cases,
imaging studies (MRI or CT scan) may be recommended to assess the depth and extent of the tumor, especially if the lesion is large or recurrent.
Early and accurate diagnosis is critical to achieving the best treatment outcomes.
Treatment Options for Dermatofibrosarcoma Protuberans
The mainstay of DFSP treatment is
complete surgical excision. Due to the tumor's locally invasive nature, surgery must ensure clear margins to prevent recurrence.
Primary Treatment Option:
- Mohs Micrographic Surgery:
- The gold standard for DFSP removal
- Offers the highest cure rate (up to
98%)
- Involves layer-by-layer removal of cancerous tissue with immediate microscopic examination of each layer
- Tissue-sparing, making it ideal for tumors located in sensitive or cosmetically important areas
Additional Treatment Options (for Certain Cases):
- Radiation Therapy:
- May be recommended after surgery in select cases to reduce recurrence risk
- Particularly useful if the tumor is large, inoperable, or located in a difficult area
- Targeted Chemotherapy (Imatinib Mesylate):
- Used for patients who cannot undergo surgery or when the tumor is considered high-risk
- May also be considered for recurrent or metastatic DFSP
- Multidisciplinary Care:
- Collaboration with medical oncologists and radiation oncologists for complex cases
Is Dermatofibrosarcoma Protuberans Curable?
Yes,
most cases of DFSP are curable, especially when treated with
Mohs micrographic surgery. Early detection and appropriate treatment are crucial in preventing recurrence and achieving long-term success.
Frequently Asked Questions About Dermatofibrosarcoma Protuberans
Is DFSP considered skin cancer?
Yes, DFSP is classified as a rare form of skin cancer, specifically a soft tissue sarcoma that originates from the dermal connective tissue.
Can DFSP spread to other organs?
While metastasis is rare, when it does occur, the lungs are the most common site. The typical course of DFSP is local invasion rather than distant spread.
Why is Mohs surgery recommended for DFSP?
Mohs micrographic surgery provides complete margin assessment, significantly lowering recurrence rates while preserving healthy tissue—making it the first-line treatment option per the National Comprehensive Cancer Network (NCCN).
Is radiation or chemotherapy always required for DFSP?
No, not always. Surgery alone is curative in most cases. Radiation therapy or chemotherapy may be considered for recurrent tumors, tumors in challenging locations, or if surgery is not an option.
How common is DFSP?
DFSP is considered very rare. Due to its complexity, treatment should be performed by a fellowship-trained Mohs surgeon with experience managing this specific tumor type.
Schedule Your DFSP Consultation Today
If you or a loved one has been diagnosed with Dermatofibrosarcoma Protuberans, trust the expertise of Dr. Ramin Fathi at Phoenix Surgical Dermatology Group. Our team specializes in advanced treatment techniques, including Mohs micrographic surgery, to provide the best possible outcomes.
Don’t wait—schedule your consultation today and take the first step toward expert care and peace of mind.
