The first 60 days: Localised adrenocortical carcinoma (ENSAT stage I to III, resectable)
Localised adrenocortical carcinoma is adrenal cortex cancer still confined to the adrenal gland and its surroundings that surgery can remove, the only treatment that cures it. Afterwards the adrenal-specific drug mitotane is given to patients whose tumour has a high risk of returning, judged by the Ki-67 index and whether it was completely removed, while low-risk patients are watched. Below, week by week, is what OnCo's record of Localised adrenocortical carcinoma (ENSAT stage I to III, resectable) 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.
Hormone work-up, contrast CT or MRI of the adrenal, chest CT and FDG-PET; no biopsy of a resectable adrenal mass; germline testing.
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.
- PathologistNamed in the standard of care for: Diagnosis and staging.
- RadiologistNamed in the standard of care for: Diagnosis and staging, After complete resection, low risk.
- SurgeonNamed in the standard of care for: Diagnosis and staging, Resectable disease, After resection, high risk, Local recurrence.
- Medical oncologistNamed in the standard of care for: Diagnosis and staging, After complete resection, low risk, After resection, high risk, Local recurrence.
- Clinical oncologist (radiotherapy)Named in the standard of care for: After resection, high risk, Local recurrence.
- Transplant and cell therapy teamNamed in the standard of care for: After resection, high risk.
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 adrenalectomy with en bloc resection of adherent structures and locoregional lymphadenectomy by an experienced surgeon; laparoscopic surgery only for small tumours; perioperative hydrocortisone for cortisol-secreting tumours.
Observation with imaging every three months (ADIUVO showed no benefit from mitotane).
Adjuvant mitotane titrated to 14 to 20 mg/L for at least two years with glucocorticoid replacement; tumour-bed radiotherapy after incomplete resection; platinum-based chemotherapy considered for very high-risk tumours (ADIUVO-2).
Repeat resection when feasible after a disease-free interval of a year or more, with mitotane; ablation or radiotherapy for small unresectable recurrences.
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 of three or more, Ki-67 index, ENSAT stage and resection status, Hormone profile, 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 ENSAT stage I to II adrenocortical carcinoma, ENSAT stage III adrenocortical carcinoma, Low-risk resected adrenocortical carcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Hormone work-up, contrast CT or MRI of the adrenal, chest CT and FDG-PET; no biopsy of a resectable adrenal mass; germline testing.
Resectable disease
- For my situation (resectable disease), which of the standard options do you recommend and why?Guideline options include: Open adrenalectomy with en bloc resection of adherent structures and locoregional lymphadenectomy by an experienced surgeon; laparoscopic surgery only for small tumours; perioperative hydrocortisone for cortisol-secreting tumours.
After complete resection, low risk
- For my situation (after complete resection, low risk), which of the standard options do you recommend and why?Guideline options include: Observation with imaging every three months (ADIUVO showed no benefit from mitotane).
- 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.
After resection, high risk
- For my situation (after resection, high risk), which of the standard options do you recommend and why?Guideline options include: Adjuvant mitotane titrated to 14 to 20 mg/L for at least two years with glucocorticoid replacement; tumour-bed radiotherapy after incomplete resection; platinum-based chemotherapy considered for very high-risk tumours (ADIUVO-2).
- Am I a candidate for Mitotane, Etoposide, Cisplatin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Local recurrence
- For my situation (local recurrence), which of the standard options do you recommend and why?Guideline options include: Repeat resection when feasible after a disease-free interval of a year or more, with mitotane; ablation or radiotherapy for small unresectable recurrences.
- 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.
Any stage
- Are there clinical trials I could join, for example of Mitotane, Cisplatin, Etoposide?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 “Whether adjuvant chemotherapy adds to mitotane in high-risk disease awaits ADIUVO-2”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Mitotane is slow to reach therapeutic levels and causes neurological and gastrointestinal toxicity in many patients”. 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.
- Localised adrenocortical carcinoma (ENSAT stage I to III, resectable): the full pageLocalised adrenocortical carcinoma is adrenal cortex cancer still confined to the adrenal gland and its surroundings that surgery can remove, the only treatment that cures it. Afterwards the adrenal-specific drug mitotane is given to patients whose tumour has a high risk of returning, judged by the Ki-67 index and whether it was completely removed, while low-risk patients are watched.
- 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.
- Adrenalectomy: Removing an adrenal gland.
- Germline vs somatic mutations: Germline mutations are inherited and in every cell; somatic mutations arise in the tumour only.
- Disease-specific staging and risk systems (FIGO, Ann Arbor, IPI, R-ISS, ELN, IMDC): Beyond the generic TNM system, gynaecological cancers (FIGO), lymphoma (Ann Arbor, IPI), myeloma (R-ISS), AML (ELN), kidney cancer (IMDC), neuroblastoma (INRG) and CLL (Rai, Binet) each have their own system that combines stage, blood tests, genetics and fitness into risk groups.
- Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
Every term links to the glossary.