The first 60 days: Dermatofibrosarcoma protuberans
Dermatofibrosarcoma protuberans is a rare, slow-growing cancer of the deeper skin that usually appears as a firm plaque or lump on the trunk and is often mistaken for a scar or cyst for years. Surgery with wide margins cures most people; for the few whose tumour cannot be removed or has spread, the pill imatinib works because almost every one is driven by a single gene fusion it blocks. Below, week by week, is what OnCo's record of Dermatofibrosarcoma protuberans says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- SurgeonNamed in the standard of care for: Localised disease, Unresectable, recurrent or metastatic disease, Neoadjuvant.
- Medical oncologistNamed in the standard of care for: Positive margins not amenable to further surgery, Unresectable, recurrent or metastatic disease, Neoadjuvant, After imatinib failure or fibrosarcomatous metastatic disease.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Positive margins not amenable to further surgery.
- Palliative and supportive care teamNamed in the standard of care for: After imatinib failure or fibrosarcomatous metastatic disease.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Wide local excision with 2 to 3 cm margins to fascia, or Mohs micrographic surgery with complete circumferential margin assessment; re-excision for involved margins.
Adjuvant radiotherapy to the tumour bed.
Imatinib for several months to shrink large or facial tumours before excision.
Imatinib after confirming the COL1A1-PDGFB fusion (EORTC 62027 and SWOG S0345); surgery after response where feasible.
- 5.After imatinib failure or fibrosarcomatous metastatic diseaseNCCN Guidelines: Dermatofibrosarcoma Protuberans
Sunitinib or pazopanib; sarcoma-type chemotherapy with doxorubicin for metastatic fibrosarcomatous disease.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example COL1A1-PDGFB fusion by fluorescence in situ hybridisation or sequencing, CD34 positivity and factor XIIIa negativity on immunohistochemistry, Fibrosarcomatous component on histology, Margin status after excision, Rare alternative PDGFD fusions in fusion-negative cases), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Classic dermatofibrosarcoma protuberans, Fibrosarcomatous DFSP, Bednar tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised disease
- For my situation (localised disease), which of the standard options do you recommend and why?Guideline options include: Wide local excision with 2 to 3 cm margins to fascia, or Mohs micrographic surgery with complete circumferential margin assessment; re-excision for involved margins.
Positive margins not amenable to further surgery
- For my situation (positive margins not amenable to further surgery), which of the standard options do you recommend and why?Guideline options include: Adjuvant radiotherapy to the tumour bed.
Unresectable, recurrent or metastatic disease
- For my situation (unresectable, recurrent or metastatic disease), which of the standard options do you recommend and why?Guideline options include: Imatinib after confirming the COL1A1-PDGFB fusion (EORTC 62027 and SWOG S0345); surgery after response where feasible.
- Am I a candidate for Imatinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Neoadjuvant
- For my situation (neoadjuvant), which of the standard options do you recommend and why?Guideline options include: Imatinib for several months to shrink large or facial tumours before excision.
- Am I a candidate for Imatinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
After imatinib failure or fibrosarcomatous metastatic disease
- For my situation (after imatinib failure or fibrosarcomatous metastatic disease), which of the standard options do you recommend and why?Guideline options include: Sunitinib or pazopanib; sarcoma-type chemotherapy with doxorubicin for metastatic fibrosarcomatous disease.
- Am I a candidate for Sunitinib, Pazopanib, Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Imatinib, Sunitinib, Pazopanib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Diagnosis is delayed for years because the tumour looks like a scar, keloid or cyst”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Responses to imatinib are not permanent and the drugs after it have only small series behind them”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Dermatofibrosarcoma protuberans: the full pageDermatofibrosarcoma protuberans is a rare, slow-growing cancer of the deeper skin that usually appears as a firm plaque or lump on the trunk and is often mistaken for a scar or cyst for years. Surgery with wide margins cures most people; for the few whose tumour cannot be removed or has spread, the pill imatinib works because almost every one is driven by a single gene fusion it blocks.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Mohs surgery: Skin cancer surgery in which the tumour is removed in thin layers, each checked under the microscope on the spot, until the edges are clear; it spares the most normal skin.
- Wide local excision: Cutting out a tumour together with a measured rim of normal-looking tissue around it, so that microscopic spread at the edge is removed too.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Every term links to the glossary.