The first 60 days: Adrenocortical carcinoma
Adrenocortical carcinoma is a rare, aggressive cancer of the adrenal gland that often over-produces hormones. Surgery is the only cure, mitotane is the one drug specific to it (with real toxicity), and chemotherapy or immunotherapy help only a minority. Below, week by week, is what OnCo's record of Adrenocortical carcinoma 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 (ENSAT I-III), Advanced, aggressive, Advanced, indolent.
- Medical oncologistNamed in the standard of care for: Localised (ENSAT I-III), Advanced, aggressive, Advanced, indolent, Progressive after chemotherapy.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised (ENSAT I-III), Advanced, indolent.
- Transplant and cell therapy teamNamed in the standard of care for: Advanced, aggressive.
- Palliative and supportive care teamNamed in the standard of care for: Advanced, aggressive.
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.
Open en bloc adrenalectomy by an experienced surgeon with locoregional lymphadenectomy; adjuvant mitotane for high-risk (Ki-67 >10%, stage III, R1) for 2-5 years; adjuvant radiotherapy for R1.
EDP-M (etoposide, doxorubicin, cisplatin + mitotane) ×6-8 with surgery for responders; streptozocin-mitotane second line.
Mitotane monotherapy (target level 14-20 mg/L) with glucocorticoid replacement; local therapies (ablation, radiotherapy) for oligometastases.
- 4.Progressive after chemotherapyNCCN category Category 2B, NCCN Guidelines: Neuroendocrine and Adrenal Tumors
Pembrolizumab, cabozantinib, gemcitabine-capecitabine; control hormone excess; clinical trials.
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 Weiss score ≥3, Ki-67 index, ENSAT stage and R status, Hormone work-up, Germline TP53, Lynch syndrome testing, Urinary steroid metabolomics), 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 Hormone-secretingvs non-functioning, Adult ACC, Paediatric ACC.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised (ENSAT I-III)
- For my situation (localised (ensat i-iii)), which of the standard options do you recommend and why?Guideline options include: Open en bloc adrenalectomy by an experienced surgeon with locoregional lymphadenectomy; adjuvant mitotane for high-risk (Ki-67 >10%, stage III, R1) for 2-5 years; adjuvant radiotherapy for R1.
- Am I a candidate for Mitotane, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, aggressive
- For my situation (advanced, aggressive), which of the standard options do you recommend and why?Guideline options include: EDP-M (etoposide, doxorubicin, cisplatin + mitotane) ×6-8 with surgery for responders; streptozocin-mitotane second line.
- Am I a candidate for Etoposide, Doxorubicin, Cisplatin or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, indolent
- For my situation (advanced, indolent), which of the standard options do you recommend and why?Guideline options include: Mitotane monotherapy (target level 14-20 mg/L) with glucocorticoid replacement; local therapies (ablation, radiotherapy) for oligometastases.
- Am I a candidate for Mitotane, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Progressive after chemotherapy
- For my situation (progressive after chemotherapy), which of the standard options do you recommend and why?Guideline options include: Pembrolizumab, cabozantinib, gemcitabine-capecitabine; control hormone excess; clinical trials.
- Am I a candidate for Pembrolizumab, Cabozantinib, 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 Cabozantinib, Pembrolizumab, Mitotane?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 “No targeted therapy despite defined genomic subgroups”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Mitotane toxicity and narrow therapeutic window”. 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.
- Adrenocortical carcinoma: the full pageAdrenocortical carcinoma is a rare, aggressive cancer of the adrenal gland that often over-produces hormones. Surgery is the only cure, mitotane is the one drug specific to it (with real toxicity), and chemotherapy or immunotherapy help only a minority.
- 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.
- Germline vs somatic mutations: Germline mutations are inherited and in every cell; somatic mutations arise in the tumour only.
- Li-Fraumeni syndrome (germline TP53): Li-Fraumeni syndrome is an inherited fault in the TP53 gene giving a lifetime cancer risk near 100% in women and ~75% in men, with sarcomas, breast cancer, brain tumours, adrenal cancer and leukaemias often in childhood.
- Hereditary cancer syndromes: About 5-10% of cancers arise from an inherited gene fault.
- 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.