Appointment sheet
Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable)
Prepared with OnCo (onco.cc/prep/advanced-adrenocortical-carcinoma/). 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 browserWhat I know so far
What is unclear to me
Changes since last time
My questions
19 on the sheetNewly diagnosed
- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example Ki-67 index and tumour burden, Hormone profile, above all cortisol, Plasma mitotane level, Mismatch repair status and tumour mutational burden, Germline TP53 and Lynch syndrome testing), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
All patients
- 5.For my situation (all patients), which of the standard options do you recommend and why?
- 6.Am I a candidate for Mitotane, and what side effects should I expect?
Low-burden, indolent disease
- 7.For my situation (low-burden, indolent disease), which of the standard options do you recommend and why?
- 8.Am I a candidate for Mitotane, and what side effects should I expect?
High-burden or progressive disease, first line
- 9.For my situation (high-burden or progressive disease, first line), which of the standard options do you recommend and why?
- 10.Am I a candidate for Etoposide, Doxorubicin, Cisplatin or related drugs, and what side effects should I expect?
After EDP-M
- 11.For my situation (after edp-m), which of the standard options do you recommend and why?
- 12.Am I a candidate for Gemcitabine, Capecitabine, Cabozantinib or related drugs, and what side effects should I expect?
Trials
- 13.For my situation (trials), which of the standard options do you recommend and why?
- 14.Am I a candidate for Relacorilant, Cabozantinib, Pembrolizumab, and what side effects should I expect?
Any stage
- 15.Are there clinical trials I could join, for example of Cabozantinib, Pembrolizumab, Relacorilant, Mitotane?
- 16.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 17.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 18.I read that “No second-line therapy has randomised evidence”. How does that affect my plan?
- 19.I read that “Most tumours are immunologically cold and cortisol excess worsens that”. How does that affect my plan?
The words I may hear
- Adrenalectomy: Removing an adrenal gland.
- Germline vs somatic mutations: Germline mutations are inherited and in every cell; somatic mutations arise in the tumour only.
- Tumour mutational burden (TMB): How many mutations a tumour has.
- Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR): Microsatellite instability is the mark of a broken DNA spell-checker (loss of MLH1, MSH2, MSH6 or PMS2) that leaves thousands of mutations, so the tumour displays abnormal proteins that T cells can recognise.
Tests and results to bring
Biomarker results to ask for: Ki-67 index and tumour burden (pace of disease), Hormone profile, above all cortisol (steroid blockade), Plasma mitotane level (14 to 20 mg/L), Mismatch repair status and tumour mutational burden (immunotherapy), Germline TP53 and Lynch syndrome testing, Molecular subgroups (CIMP-high, C1A) as prognostic markers in research.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- All patients: Mitotane from diagnosis with glucocorticoid replacement; adrenal enzyme inhibitors (metyrapone, osilodrostat, ketoconazole) for cortisol excess; thromboprophylaxis and infection vigilance. (Mitotane)
- Low-burden, indolent disease: Mitotane alone, with resection, thermal ablation or stereotactic radiotherapy of limited metastases. (Mitotane, Thermal ablation (RFA, microwave, cryo), SBRT / SABR (stereotactic radiotherapy), Adrenalectomy)
- High-burden or progressive disease, first line: Etoposide, doxorubicin and cisplatin with mitotane (EDP-M, FIRM-ACT). (Etoposide, Doxorubicin, Cisplatin, Mitotane, Cytotoxic chemotherapy)
- After EDP-M: Gemcitabine with capecitabine; cabozantinib; pembrolizumab, especially for mismatch-repair-deficient tumours; streptozocin-mitotane; clinical trials. (Gemcitabine, Capecitabine, Cabozantinib, Pembrolizumab, Immune checkpoint inhibitors, Small-molecule kinase inhibitors)
- Trials: Relacorilant with checkpoint inhibition for cortisol-secreting tumours; cabozantinib with immunotherapy; radiolabelled CYP11B tracers. (Relacorilant, Cabozantinib, Pembrolizumab)
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 browserWhat I was told
Agreed next steps, dates and who to call