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Living with cancer· Appointment prep

Appointment sheet: Dermatofibrosarcoma protuberans

One page to bring and write on: your details, the questions for Dermatofibrosarcoma protuberans plus your own, the words you may hear, what to bring, the treatments the standard of care names, and room for the answers and agreed next steps. What you type stays in this browser. Print it or save it as a PDF. New to all this? Start with the first 60 days. Orientation, not medical advice.

Tick the questions to print

All of this cancer's questions start ticked. Untick what does not apply; ticks are kept in this browser. .

Your own questions

Shared with the prep pack, so questions you add there appear here too.

Print or save as PDF

Use (or Ctrl+P, Cmd+P on a Mac). To keep a copy, choose Save as PDF as the destination in the print dialog. Only the sheet prints; the controls stay on screen. Your typed notes print where you typed them; empty fields print as ruled lines to write on.

Appointment sheet

Dermatofibrosarcoma protuberans

Prepared with OnCo (onco.cc/prep/dermatofibrosarcoma-protuberans/). Orientation, not medical advice; your team knows your case.

My details

Name
Date of appointment
Hospital and clinician
Who is coming with me

What I know, what is unclear, changes to discuss

Saved in this browser
What I know so far
What is unclear to me
Changes since last time

My questions

17 on the sheet
Newly diagnosed
  1. 1.What is my exact diagnosis, stage, and grade, and which tests established them?
  2. 2.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?
  3. 3.Which subtype is my cancer, and does that change the recommended treatment?
  4. 4.Is germline (inherited) genetic testing recommended for me or my family?
Localised disease
  1. 5.For my situation (localised disease), which of the standard options do you recommend and why?
Positive margins not amenable to further surgery
  1. 6.For my situation (positive margins not amenable to further surgery), which of the standard options do you recommend and why?
Unresectable, recurrent or metastatic disease
  1. 7.For my situation (unresectable, recurrent or metastatic disease), which of the standard options do you recommend and why?
  2. 8.Am I a candidate for Imatinib, and what side effects should I expect?
Neoadjuvant
  1. 9.For my situation (neoadjuvant), which of the standard options do you recommend and why?
  2. 10.Am I a candidate for Imatinib, and what side effects should I expect?
After imatinib failure or fibrosarcomatous metastatic disease
  1. 11.For my situation (after imatinib failure or fibrosarcomatous metastatic disease), which of the standard options do you recommend and why?
  2. 12.Am I a candidate for Sunitinib, Pazopanib, Doxorubicin, and what side effects should I expect?
Any stage
  1. 13.Are there clinical trials I could join, for example of Imatinib, Sunitinib, Pazopanib?
  2. 14.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
  3. 15.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
  4. 16.I read that “Diagnosis is delayed for years because the tumour looks like a scar, keloid or cyst”. How does that affect my plan?
  5. 17.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?

The words I may hear

  • 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.

Tests and results to bring

Biomarker results to ask for: COL1A1-PDGFB fusion by fluorescence in situ hybridisation or sequencing (required before imatinib), CD34 positivity and factor XIIIa negativity on immunohistochemistry, Fibrosarcomatous component on histology, Margin status after excision, Rare alternative PDGFD fusions in fusion-negative cases.

Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.

The treatments I may be offered

  • Localised disease: 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. (Mohs surgery, Wide local excision)
  • Positive margins not amenable to further surgery: Adjuvant radiotherapy to the tumour bed. (IMRT / IGRT (modern external beam))
  • Neoadjuvant: Imatinib for several months to shrink large or facial tumours before excision. (Imatinib)
  • Unresectable, recurrent or metastatic disease: Imatinib after confirming the COL1A1-PDGFB fusion (EORTC 62027 and SWOG S0345); surgery after response where feasible. (Imatinib, PDGFRB)
  • After imatinib failure or fibrosarcomatous metastatic disease: Sunitinib or pazopanib; sarcoma-type chemotherapy with doxorubicin for metastatic fibrosarcomatous disease. (Sunitinib, Pazopanib, Doxorubicin)

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.

Answers and next steps

Saved in this browser
What I was told
Agreed next steps, dates and who to call