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Living with cancer· Appointment prep

Appointment sheet: Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms

One page to bring and write on: your details, the questions for Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms 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

Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms

Prepared with OnCo (onco.cc/prep/histiocytoses/). 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 BRAF V600E, MAP2K1, ARAF, NRAS, KRAS, PIK3CA, RAF1 and ALK fusions on NGS, Immunophenotype: CD68, CD163, factor XIIIa positive; CD1a and langerin negative; S100 with emperipolesis, FDG-PET/CT for extent and response, Cardiac MRI and brain MRI for organ involvement), 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?
ECD, BRAF V600E-mutant, needing treatment
  1. 5.For my situation (ecd, braf v600e-mutant, needing treatment), which of the standard options do you recommend and why?
  2. 6.Am I a candidate for Vemurafenib, Dabrafenib + trametinib, and what side effects should I expect?
ECD or RDD, BRAF wild-type or intolerant of BRAF inhibitors
  1. 7.For my situation (ecd or rdd, braf wild-type or intolerant of braf inhibitors), which of the standard options do you recommend and why?
  2. 8.Am I a candidate for Cobimetinib, Interferon alfa-2a/2b, and what side effects should I expect?
Rosai-Dorfman disease
  1. 9.For my situation (rosai-dorfman disease), which of the standard options do you recommend and why?
  2. 10.Am I a candidate for Cladribine, Cobimetinib, and what side effects should I expect?
Histiocytic sarcoma
  1. 11.For my situation (histiocytic sarcoma), which of the standard options do you recommend and why?
  2. 12.Am I a candidate for Cyclophosphamide, Doxorubicin, Etoposide or related drugs, and what side effects should I expect?
Any stage
  1. 13.Are there clinical trials I could join, for example of Cobimetinib, Vemurafenib, Dabrafenib + trametinib?
  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 “Most patients relapse when BRAF or MEK inhibitors stop; intermittent dosing and ctDNA-guided discontinuation are being tested”. How does that affect my plan?
  5. 17.I read that “Neurodegenerative ECD and LCH do not reverse with targeted therapy; earlier treatment and neuroprotective trials are the response”. How does that affect my plan?

The words I may hear

  • Driver mutation: One of the few mutations in a tumour that actually causes it to grow.
  • BRAF V600E mutation: A single spelling change in the BRAF gene that jams a growth switch permanently on.
  • Circulating tumour DNA (ctDNA): Circulating tumour DNA (ctDNA) consists of fragments of DNA shed by tumour cells into the blood, detectable with sensitive sequencing.
  • Retroperitoneum: The space at the back of the abdomen, behind the gut's lining, holding the kidneys, adrenals, pancreas, aorta and the para-aortic lymph nodes.

Tests and results to bring

Biomarker results to ask for: BRAF V600E (tissue; plasma ctDNA for monitoring), MAP2K1, ARAF, NRAS, KRAS, PIK3CA, RAF1 and ALK fusions on NGS, Immunophenotype: CD68, CD163, factor XIIIa positive; CD1a and langerin negative (ECD, RDD); S100 with emperipolesis (RDD), FDG-PET/CT for extent and response, Cardiac MRI and brain MRI for organ involvement, Clonal haematopoiesis (mutations shared with myeloid clones in some patients).

Scans and tests linked to this cancer: Comprehensive genomic profiling, FDG PET, Liquid biopsy (ctDNA), MRI.

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

The treatments I may be offered

  • ECD, BRAF V600E-mutant, needing treatment: Vemurafenib (FDA approval 2017) or dabrafenib, often with a MEK inhibitor to reduce toxicity; long-term treatment at the lowest effective dose. (Vemurafenib, Dabrafenib + trametinib, BRAF)
  • Rosai-Dorfman disease: Observation for asymptomatic nodal disease; surgery for isolated masses; steroids, sirolimus, cladribine or MEK inhibitors for symptomatic or multifocal disease. (Cladribine, Cobimetinib)
  • Histiocytic sarcoma: Lymphoma-type chemotherapy (CHOP, ICE, or similar), radiotherapy for localised disease, MAPK-pathway inhibitors where mutations are found; clinical trials. (Cyclophosphamide, Doxorubicin, Etoposide, Cobimetinib)
  • ECD or RDD, BRAF wild-type or intolerant of BRAF inhibitors: Cobimetinib (FDA approval October 2022 for histiocytic neoplasms) or another MEK inhibitor; interferon alfa or pegylated interferon as an alternative. (Cobimetinib, Interferon alfa-2a/2b)

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