ACC is a malignant adrenal-cortex tumor requiring cancer and hormone assessment. Definition.
- A hormone-producing tumor and a nonfunctional tumor can both be ACC.
- Specialists determine assessment and care.
- Possible adrenal crisis requires immediate emergency help.
Table of contents
- Evidence summary
- Symptoms and assessment
- Hormones, spread and pathology
- Specialist cancer care
- Nutrition, emotional support and supplement limits
- What the evidence establishes
- Treatment safety
- Medicine review
- Pregnancy and breastfeeding
- Planning and follow-up
- Animal and laboratory evidence boundaries
- Funding and source roles
- Frequently asked questions
- Sources and funding notes
Evidence summary
Clinical descriptions, care guidance and independently established treatment outcomes have different evidentiary roles. The table identifies what the reviewed sources can support and which financial or clinical questions remain unresolved.
| Question / approach | Evidence reviewed | Funding / conflicts | Interpretation / limits |
|---|---|---|---|
| Diagnosis | Imaging, endocrine and pathology assessment | Incomplete source clearance | No self-diagnosis |
| Expert care | Dated guidance | Author interests | No cleared drug ranking |
| Mitotane labels | EU/US scope and warnings | Maker-produced | Safety context |
| ADIUVO | Selected postoperative population | HRA drug/monitoring supply | Efficacy excluded |
| DLK1 models | Cells/animals | ADC study funding | No patient-benefit inference |
Symptoms and assessment
ACC begins in the outer adrenal cortex. These glands above the kidneys produce hormones affecting blood pressure, salt balance, metabolism and sexual development. Functional tumors produce excess hormones; nonfunctional does not mean benign. Inner-medulla tumors, including pheochromocytoma, are different. Adrenal identity.
Possible symptoms include abdominal or back pain, a lump or a feeling of fullness. Some nonfunctional tumors have no early symptoms. Excess cortisol can cause muscle weakness, high blood pressure or high blood sugar; excess aldosterone may cause weakness or cramps. Changes related to sex hormones can affect menstruation, hair growth or sexual characteristics. These changes have other possible causes and need clinical assessment. Symptoms.
Assessment may combine imaging such as CT or MRI with blood or urine hormone tests. The purpose is to characterize the mass, hormone production and possible spread. Prognosis depends on several features, including stage, whether complete removal is possible and general health; a scan finding alone cannot supply an individual outlook. Diagnostic context.
Children and other adrenal entities need separate pathways.
Hormones, spread and pathology
Stage describes where the cancer is, rather than how much hormone it produces. Early stages are confined to the adrenal gland; regional disease can involve nearby tissues, nodes or major blood vessels. Distant disease has spread elsewhere. An ACC deposit in the lung remains metastatic ACC, rather than becoming a new primary lung cancer. Recurrence means cancer has returned after treatment. Staging.
Specialists interpret cortical origin (SF1), malignancy (Weiss criteria), proliferation (Ki67), stage and margins. Pathology guidance.
Inherited-risk testing examines germline changes; tumor testing addresses changes in the cancer. They answer different questions. Genetic counseling helps interpret the personal and family implications, and an uncertain variant usually does not determine clinical care by itself. Ask whether the family history or diagnosis warrants referral, rather than assuming every tumor result establishes an inherited syndrome. Genetic-testing distinctions.
Specialist cancer care
Suspected ACC should not automatically undergo needle biopsy: it may compromise curative surgery. Selected biopsy needs specialist review, exclusion of pheochromocytoma and a result that changes care. An incidental adrenal mass is not a cancer diagnosis. Biopsy boundary.
An expert team with an adrenal cancer surgeon aims for intact removal when feasible. Postoperative mitotane depends on recurrence risk. Expert care.
Stage-dependent care can include adrenal surgery, selected treatment of recurrent or advanced disease, radiation and systemic medicines. Treatment goals may include removal, disease control or symptom relief. The appropriate combination depends on the actual disease setting; online stage summaries do not establish that an operation is feasible for a particular person. Treatment settings.
Radiation targets a planned treatment area and may also be used to relieve cancer-related symptoms. Planning and the tissues exposed matter. A local treatment does not treat every distant deposit at once; discuss what its proposed role is in the overall plan. Radiation context.
Nutrition, emotional support and supplement limits
Cancer or treatment can change appetite, taste, digestion and the ability to eat enough. A dietitian can assess symptoms, weight, usual intake and treatment needs, then tailor protein and energy support. Report difficulty eating or ongoing weight loss. A restrictive anticancer diet should not replace this assessment, and the same food or calorie plan is not suitable for everyone. Nutrition assessment.
Distress can accompany diagnosis and treatment. Discuss practical costs, sleep and emotional concerns with the team; counseling, social-work help or support groups may be useful. Physical symptoms attributed to anxiety still need review for medicine or treatment causes. Support is care in its own right, not a promised effect on cancer survival. Supportive care.
Complementary products must not replace or delay cancer treatment. Supplements may interfere with treatment, and a natural label does not establish safety. Review any intended product with the oncology team. Supplement safety.
No supplement receives an independent ACC-treatment recommendation here. This is the result of the review’s evidence screen, not a claim that every possible product has been tested.
What the evidence establishes
Current NCI patient education describes surgery, radiation, medicines and clinical trials as treatment approaches. It gives care context; it does not provide a conflict-cleared ranking of drug benefits for this individual rare cancer. Patient treatment overview.
The professional PDQ is an evidence summary, not NCI policy or a guideline. Lead Jaydira del Rivero’s commercial research connection is traced separately. Professional source role.
ADIUVO studied postoperative adults with completely resected, selected low-to-intermediate-risk ACC. It was open-label and stopped early because recruitment was slow. HRA supplied mitotane and free monitoring. Its outcomes therefore do not enter this review’s independent efficacy verdict; the selected population also cannot represent every recurrence-risk group. Trial scope and support.
Clinical trials ask whether a proposed intervention works and whether it is safe. Participation or a trial listing is not proof of benefit. Ask about eligibility, sponsor, alternatives and responsibility for care and costs before deciding whether a study is suitable. Trial context.
Recognized clinical pathways can be explained with their funding limits. That is different from asserting an independently proven comparative survival benefit. This guide gives no numerical personal prognosis or unsupported drug-superiority claim.
Treatment safety
Surgery can cause bleeding, infection, pain, damage to nearby tissues and anesthesia reactions. Obtain instructions for the actual operation and recovery, including activity limits, wound care and whom to contact. Report uncontrolled pain or suspected infection rather than applying an unrelated operation’s discharge instructions. Surgical safety.
Chemotherapy can affect healthy cells as well as cancer cells, causing problems such as nausea, fatigue, mouth soreness or hair loss. Side effects do not show whether treatment is working; the clinical team uses appropriate response assessments. Systemic-treatment effects.
Contact the clinician immediately for illness or injury on mitotane. Shock, severe trauma or infection require temporarily withholding mitotane and giving steroids under medical care. Clinicians monitor neurologic, liver and blood toxicity; report pelvic pain or vaginal bleeding immediately. US label.
For known adrenal insufficiency, rapidly worsening weakness, severe abdominal pain or vomiting, confusion, seizures or loss of consciousness can indicate crisis. Seek immediate emergency help, even if prescribed emergency steroid treatment has already been given. The NHS uses 999; elsewhere use the local emergency service. Adrenal-crisis emergency.
During cancer treatment, fever, chills or other infection signs require immediate contact with the oncology team. Infections can be life-threatening. Ask before using fever-reducing medicine, which may mask a serious problem. Follow the team’s urgent-care instructions. Infection warning.
Medicine review
Avoid spironolactone, warfarin, certain CYP3A medicines and hormonal contraceptives with mitotane; unavoidable warfarin needs prescriber monitoring. Do not change medicines yourself. US label.
The EU label warns about enzyme induction, St John’s wort interactions and altered hormone-binding laboratory results. Do not drive or use machinery. Specialists monitor blood levels; elimination remains slow after stopping. EU monitoring and driving.
Pregnancy and breastfeeding
The US label says to verify pregnancy status. It advises effective nonhormonal contraception and no breastfeeding during treatment and until mitotane levels become undetectable afterward. Fetal harm is possible. US label.
The EU label excludes breastfeeding and concomitant spironolactone, and warns about pregnancy. These restrictions require the treating clinician’s plan, including the period after stopping. EU restrictions.
Planning and follow-up
The EU indication is symptomatic advanced unresectable, metastatic or relapsed ACC; its effect in nonfunctioning tumors is not established. EU licensed scope.
The US label includes inoperable functional and nonfunctional ACC. US scope.
The EMA portal posted product information on 11 March 2026; its older overview is not the current prescribing document. Authorization does not determine individual treatment. Current-document identification.
Follow-up care should name the responsible team, planned assessments and problems to report. New or persistent symptoms should be raised without waiting for the next scheduled visit. Late effects and other health needs also matter after treatment. Keep a treatment summary and share it with clinicians involved in ongoing care. Follow-up planning.
Animal and laboratory evidence boundaries
A 2025 DLK1 study used cell and animal models. ADC Therapeutics supported the study, and Roper and del Rivero explicitly declared its research funding. Laboratory activity cannot establish patient benefit, a safe regimen or an approved ACC treatment. Original model and funding record.
Models cannot establish a patient regimen, approved ACC treatment or comparative product benefit.
Funding and source roles
Research funding at a glance
39 disclosure entries. The counts below summarize independence tiers explicitly assigned in this article. They count disclosures, not studies, funding amounts or evidence quality.
Consult this article’s source and funding notes for named funders, countries, relationships and exceptions where available. Institutional backing, researcher interests and trial sponsorship are separate questions. Public funding alone does not establish independence; commercial ties alone do not prove a claim false. This overview is not a new financial audit.
The source-specific map separates documented institutional funding from disease-page payments and trial sponsorship. Unknown allocations remain unknown. A public agency, charity or academic address does not by itself establish independent treatment efficacy.
| Source | Funding / backers | Country / jurisdiction | Independence | Credibility / incentives / gaps |
|---|---|---|---|---|
| NCI: what is ACC | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI: ACC symptoms | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI: ACC diagnosis | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI: ACC stages | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI: ACC treatment | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI: ACC treatment by stage | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 14 February 2025; education, not conflict-cleared ACC outcomes. |
| NCI professional ACC PDQ | Lead del Rivero has documented ADC Therapeutics study support; PDQ payments unclosed. | United States | Tier 3 — connected reviewer | C provisional. Updated 13 December 2024; older treatment literature, not guideline or cleared comparative efficacy. |
| ESE–ENSAT ACC guideline (2018) | ESE/ENSAT support; HRA/other company research, advisory and speaker ties. Current receipts unclosed. | Lead Würzburg, Germany; international authors | Tier 3 — connected authors | C provisional. Final 2018 guidance; no independent efficacy clearance. |
| ESE incidentaloma guideline, 2023 | ESE sponsorship; ENSAT and COST HARMONISATION CA20122 support. Author Table S1 unavailable. | Lead Würzburg, Germany; international authors | Tier 3 — incompletely cleared guideline | C provisional. Final DOI 10.1093/ejendo/lvad066; incidental-mass scope. Missing S1 limits financial assessment. |
| ADIUVO original trial, 2023 | AIFA, EU FP7, DFG, CRUK and French ministry support; HRA supplied mitotane/free Lysosafe. Terzolo/Berruti declare HRA interests. | Lead Turin, Italy; seven-country trial; HRA Paris, France | Tier 4 — manufacturer-supplied trial | D for independence. Open-label, early-stopped selected-risk trial; outcomes excluded from independent verdict. |
| DLK1 original laboratory study, 2025 | NIH, DOD and other public support plus ADC Therapeutics. Roper/del Rivero declare company funding for this study. | Lead NCI, Bethesda, United States | Tier 4 — manufacturer-supported study | D for independence. 1 July 2025; cell/animal models, not established patient benefit or PDQ payment proof. |
| EU Lysodren product information | Esteve-produced; product interests and preparation payments unclosed. | EU licence; holder Barcelona, Spain | Tier 4 — maker-produced label | D independence; safety context, not cleared efficacy. |
| US Lysodren label | Esteve label; payments unclosed. | US; Esteve Barcelona, Spain; manufacturer Italy | Tier 4 — maker-produced label | D independence; warnings. May 2026 body; March header; August archive. |
| EMA Lysodren portal | Regulatory record; product-specific agency receipts and assessor interests unclosed. | European Union | Tier 3 — fee-supported regulator | C provisional. PI posted 11 March 2026; 2013 overview is older. Authorization does not clear independent efficacy. |
| NCI genetic testing fact sheet | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Reviewed 18 April 2024; education, not conflict-cleared ACC outcomes. |
| NCI nutrition during treatment | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Posted 15 October 2024; education, not conflict-cleared ACC outcomes. |
| NCI emotions and cancer | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 9 April 2025; education, not conflict-cleared ACC outcomes. |
| NCI cancer surgery | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Reviewed 8 November 2024; education, not conflict-cleared ACC outcomes. |
| NCI radiation therapy | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 15 May 2025; education, not conflict-cleared ACC outcomes. |
| NCI chemotherapy | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Reviewed 15 May 2025; education, not conflict-cleared ACC outcomes. |
| NCI infection during cancer care | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | C provisional. Reviewed 23 January 2020; education, not conflict-cleared ACC outcomes. |
| NCI follow-up care | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 2 December 2024; education, not conflict-cleared ACC outcomes. |
| NCI clinical-trial explainer | Page, contributor and underlying-study financial allocations unclosed. | United States | Tier 2 — public clinical context | B provisional. Updated 3 November 2024; education, not conflict-cleared ACC outcomes. |
| NHS Addison’s disease: crisis | Specific page and contributor allocations unclosed. | United Kingdom | Tier 2 — public safety context | B provisional. Reviewed 19 September 2025; adrenal-insufficiency emergency context, not ACC diagnosis. |
| NCCIH cancer complementary approaches | Acknowledged 2021 expert reviewers; current personal/page allocations unclosed. | United States | Tier 2 — public safety context | C provisional. October 2021; nonreplacement and interference, not independent ACC product efficacy. |
| NCI institutional budget | Congressional appropriations through NIH/HHS. Exact page/project allocation unclosed. | Bethesda, Maryland, United States | Tier 3 — financial self-report | B provisional. Updated 14 May 2026; enacted budgets differ from requests. No underlying-study clearance. |
| NCI gift fund | Conditional/unconditional donations permitted; recipient projects and current donors not fully traced. | Bethesda, Maryland, United States | Tier 3 — financial self-report | B provisional. August 2025; permission is not proof of a specific page donation. |
| NCI PDQ editorial process | Nongovernment board honoraria/travel; government members not paid honoraria. Individual receipts unclosed. | United States | Tier 3 — process self-report | B provisional. 1 November 2022; editorial autonomy/recusal do not clear author finance. |
| ESE 2025 accounts | Member/corporate, sponsor/grant, publishing, congress and investment income; pharmaceutical support. | Registered Bristol, England, United Kingdom | Tier 3 — financial self-report | B provisional. Selected income, notes and identity read; exact guideline allocations/current author payments unclosed. |
| EMA 2025 annual report | Fees and EU contributions; selected annual finance and address sections. | Amsterdam, Netherlands; EU agency | Tier 3 — financial self-report | B provisional. 2025 actual revenue distinguished from projections; product allocations/assessor receipts unclosed. |
| EMA June 2025 anti-fraud strategy | Agency says it charges pharmaceutical companies for scientific procedures/services. | European Union | Tier 3 — institutional process self-report | B provisional. Selected page 7; funding forecasts are not actual annual receipts or independence proof. |
| NCCIH appropriations history | Congressional appropriations; displayed series through FY2024. | United States | Tier 3 — financial self-report | B provisional. A newer site footer does not establish newer figures; page allocation unclosed. |
| NCCIH donation policy | Conditional/unconditional gifts permitted; exact donors and clinical-page allocation unclosed. | Bethesda, Maryland, United States | Tier 3 — financial self-report | B provisional. Gift authority is not evidence that a named company paid this page. |
| NHS national content policy | DHSC site funding; no advertising/corporate sponsorship stated. | United Kingdom | Tier 3 — process self-report | C provisional. October 2022; review due October 2025 passed. Not provider finances or page-specific contributor clearance. |
| ADC Therapeutics 2025 Form 10-K | Product/licensing/royalty revenue; Oaktree/Owl Rock lending and HCR royalty finance. | Epalinges, Switzerland; US listing | Tier 4 — company-produced finance | D self-interest. Filed March 2026; selected business/finance sections. ZYNLONTA finance is not ADCT-701 allocation; current full ownership unclosed. |
| Esteve completed HRA acquisition | Completed purchase from Perrigo includes Lysodren; fixed/contingent consideration is not cash paid. | Spain | Tier 4 — company-produced announcement | D self-interest. 10 July 2024; territorial payments and beneficial ownership unclosed. |
| Esteve FY2025 results announcement | Pharma/CDMO sales; management report, not full audited accounts. | Barcelona, Spain | Tier 4 — company-produced finance | D self-interest. 30 April 2026; product allocation, debt and ultimate backers unclosed. |
| Esteve FY2023 historical report | Lubea minority entry: 26% in 2023. | Barcelona, Spain | Tier 4 — company-produced finance | D self-interest. 16 May 2024; historical, not current cap table. |
| Esteve 2023 shareholder announcement | German private investor Lubea; Grupo Esteve Lifesciences majority; transaction then pending. | Spain; German investor described | Tier 4 — company-produced announcement | D self-interest. 17 May 2023; later report records entry, not present percentages. |
Frequently asked questions
Does nonfunctional ACC mean it is benign? No. It describes hormone production, not benign behavior.
Does every adrenal mass require a biopsy? No. Specialist assessment is essential; suspected ACC may need a different pathway, and pheochromocytoma must be excluded before selected biopsy.
Will everyone need mitotane after surgery? Not automatically. Specialists assess recurrence risk.
What needs urgent help? Possible adrenal crisis requires immediate emergency assessment.
Does a tumor gene result prove inherited risk? Not by itself. Germline and tumor testing answer different questions; genetic counseling helps interpret which assessment is appropriate.
Sources and funding notes
The final 2018 guideline used its author’s mirror.
The final 2023 guideline used a public mirror; its author-disclosure Table S1 remained inaccessible.
NCI institutional finance, gifts and editorial process appear once as separate profiles. They do not close individual page, reviewer or trial allocations.
Selected financial sections were read, not audited; historical ownership does not establish current ownership.
- NCI: what is ACC — Cortex, functional status and distinct adrenal entities.
- NCI: ACC symptoms — Mass symptoms and hormone-related changes.
- NCI: ACC diagnosis — Imaging, hormone evaluation and prognosis context.
- NCI: ACC stages — Local, regional, distant and recurrent disease.
- NCI: ACC treatment — Surgery, radiation, medicines and trial context.
- NCI: ACC treatment by stage — Stage-dependent treatment settings.
- NCI professional ACC PDQ — Professional context and named lead.
- ESE–ENSAT ACC guideline (2018) — Expert care and pathology.
- ESE incidentaloma guideline, 2023 — Selected assessment and biopsy recommendations.
- ADIUVO original trial, 2023 — Population, limitations and exact support disclosures.
- DLK1 original laboratory study, 2025 — Preclinical boundary and reviewer research connection.
- EU Lysodren product information — EU indication, restrictions, monitoring and driving.
- US Lysodren label — US warnings.
- EMA Lysodren portal — Holder and current-document identification.
- NCI genetic testing fact sheet — Germline versus tumor testing, counseling and uncertainty.
- NCI nutrition during treatment — Dietitian assessment and individualized intake.
- NCI emotions and cancer — Counseling and practical support; no survival claim.
- NCI cancer surgery — Preparation, surgical risks and recovery.
- NCI radiation therapy — Local treatment, planning and palliation.
- NCI chemotherapy — Systemic treatment and healthy-cell effects.
- NCI infection during cancer care — Urgent oncology contact and fever-masking warning.
- NCI follow-up care — Individual follow-up plans and new symptoms.
- NCI clinical-trial explainer — Research questions, not guaranteed benefit.
- NHS Addison’s disease: crisis — Rapid deterioration and emergency action.
- NCCIH cancer complementary approaches — Supplement and complementary-treatment boundaries.
- NCI institutional budget — Institutional public finance.
- NCI gift fund — Separate donation route.
- NCI PDQ editorial process — Board process, not clinical guideline endorsement.
- ESE 2025 accounts — Institutional funding routes, not whole-account clearance.
- EMA 2025 annual report — Printed pages 78–79 and contact, not complete financial audit.
- EMA June 2025 anti-fraud strategy — Industry-fee route only.
- NCCIH appropriations history — Historical public finance.
- NCCIH donation policy — Separate gift route.
- NHS national content policy — National website policy only.
- ADC Therapeutics 2025 Form 10-K — Company funding routes, not efficacy.
- Esteve completed HRA acquisition — Dated transaction identity.
- Esteve FY2025 results announcement — Current business revenue routes.
- Esteve FY2023 historical report — Dated shareholder fact.
- Esteve 2023 shareholder announcement — Historical backer identity and pending-versus-completed boundary.
Educational research reviewed 5 October 2026. Diagnosis and treatment require a qualified clinician; this article does not provide an individual prescription or replace urgent assessment.
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