Overview
Dermatofibrosarcoma protuberans, often called DFSP, is a rare type of skin cancer that starts in the deep layers of the skin. It usually appears as a firm, raised patch or lump on the skin that grows slowly over months or years. DFSP most often shows up on the trunk (chest, back, or abdomen), but it can also appear on the arms, legs, head, or neck. The tumor is made up of abnormal cells that grow in a web-like pattern, which allows them to spread into nearby tissue and makes them tricky to remove completely. The most important thing to know about DFSP is that it very rarely spreads to other parts of the body (metastasizes), but it has a strong tendency to grow back after surgery if it is not removed with wide enough margins. Early on, DFSP can look like a harmless scar, bruise, or skin discoloration, which is why it is often mistaken for something less serious and may go undiagnosed for years. Treatment usually involves surgery to remove the tumor with a large border of healthy tissue around it. A specialized surgical technique called Mohs micrographic surgery is often preferred because it removes the tumor layer by layer while checking the edges under a microscope, reducing the chance of it coming back. For tumors that cannot be fully removed with surgery, or that have spread, a targeted drug called imatinib (Gleevec) is FDA-approved and works well for most patients. The overall outlook for DFSP is generally good when it is caught and treated early.
Also known as:
Key symptoms:
A firm, raised lump or nodule on the skinA flat, discolored patch of skin that looks like a scar or bruiseSlow-growing skin growth that may have been present for yearsSkin that feels hard or rubbery to the touchA bump that may be skin-colored, reddish, brownish, or purplishTenderness or mild pain around the growthA growth that keeps coming back after being removedSkin that appears puckered or dimpled around the lumpGradual increase in the size of a skin lesion over time
Clinical phenotype terms (8)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
2 eventsOslo University Hospital — PHASE2
The Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College — PHASE1
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
3 availableImatinib Mesylate
Adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP).
Imkeldi
Adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP).
Imatinib Mesylate
Adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP)
Clinical Trials
View all trials with filters →No actively recruiting trials found for Dermatofibrosarcoma protuberans at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Dermatofibrosarcoma protuberans.
Community
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Start the conversation →Latest news about Dermatofibrosarcoma protuberans
Disease timeline:
New recruiting trial: Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma.
A new clinical trial is recruiting patients for Dermatofibrosarcoma protuberans
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Was my tumor completely removed, and what were the surgical margins?,What is my risk of this tumor coming back, and how will we monitor for that?,Should I have Mohs surgery, and is that available at this center?,Does my tumor have the COL1A1-PDGFB gene fusion, and does that affect my treatment options?,How often do I need follow-up appointments, and what should I watch for between visits?,If the tumor comes back, what are my treatment options?,Are any of my family members at increased risk for this condition?
Common questions about Dermatofibrosarcoma protuberans
What is Dermatofibrosarcoma protuberans?
Dermatofibrosarcoma protuberans, often called DFSP, is a rare type of skin cancer that starts in the deep layers of the skin. It usually appears as a firm, raised patch or lump on the skin that grows slowly over months or years. DFSP most often shows up on the trunk (chest, back, or abdomen), but it can also appear on the arms, legs, head, or neck. The tumor is made up of abnormal cells that grow in a web-like pattern, which allows them to spread into nearby tissue and makes them tricky to remove completely. The most important thing to know about DFSP is that it very rarely spreads to other p
How is Dermatofibrosarcoma protuberans inherited?
Dermatofibrosarcoma protuberans follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Dermatofibrosarcoma protuberans typically begin?
Typical onset of Dermatofibrosarcoma protuberans is adult. Age of onset can vary across affected individuals.
Which specialists treat Dermatofibrosarcoma protuberans?
25 specialists and care centers treating Dermatofibrosarcoma protuberans are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.