Adrenal cortex carcinoma (ACC) is a rare and aggressive cancer that starts in the outer layer (cortex) of the adrenal gland. Each person has two adrenal glands above the kidneys. The adrenal cortex makes important hormones, such as cortisol, aldosterone, and sex hormones (androgens and estrogens). In ACC, cells grow out of control and may overproduce these hormones. Some tumors “function” (they make extra hormones), and others are “non-functioning.” The cancer can grow nearby, spread to lymph nodes, or travel to other organs like the liver or lungs. Early diagnosis and treatment improve outcomes.
Adrenal cortex carcinoma (ACC) is a rare cancer that starts in the outer layer of the adrenal gland. The adrenal cortex makes important hormones, like cortisol (stress hormone), aldosterone (salt and blood-pressure control), and androgens (sex hormones). In ACC, the tumor can grow fast, can spread to nearby tissues or far organs, and may release too much hormone. Too much cortisol can cause Cushing’s syndrome (weight gain, high blood pressure, diabetes, weak bones). Too much aldosterone can cause very high blood pressure and low potassium. Too much androgens can cause hair growth or other changes. The most important treatment for tumors that can be removed safely is complete surgery with an open approach, because it gives the best chance to control the disease. Medicines like mitotane and combination chemotherapy may be used when the cancer has spread, cannot be fully removed, or after surgery in higher-risk cases. Immunotherapy helps only a small group of patients so far, and clinical trials are important for many people with ACC. Cancer.govPubMed
Other names
ACC is also called adrenocortical carcinoma, adrenal cortical carcinoma, carcinoma of the adrenal cortex, malignant adrenal cortical tumor, suprarenal cortical carcinoma, or simply adrenal cortex cancer. It is different from tumors of the adrenal medulla (like pheochromocytoma). Doctors may say “functional ACC” when the tumor makes excess hormones (such as cortisol or androgens), and “non-functional ACC” when it does not. In children, it is sometimes called pediatric adrenocortical carcinoma.
Types
By hormone activity:
Functional ACC produces too much hormone. This can cause Cushing syndrome (extra cortisol), Conn syndrome (extra aldosterone), virilization (too much androgen), or feminization (too much estrogen). Non-functional ACC does not make excess hormone and often presents with pain or a lump.
By age group:
Pediatric ACC is seen in children, often linked to inherited gene changes. Adult ACC is more common in adults in their 40s–60s.
By spread (clinical stage concept):
Localized (only in the adrenal), locally advanced (into nearby tissues or nodes), and metastatic (spread to distant organs).
By origin:
Sporadic (no known family link) and hereditary-associated (occurs with inherited syndromes like Li-Fraumeni or Beckwith–Wiedemann).
Causes
Note: For most people, the exact cause is unknown. Below are well-described risk factors and biologic mechanisms that can contribute to ACC. Each item explains the idea in plain words.
Li-Fraumeni syndrome (TP53 mutation).
This inherited DNA repair problem weakens the body’s natural “tumor-police” (p53). When p53 is broken, damaged adrenal cells can survive and become cancer.Beckwith–Wiedemann syndrome (11p15 imprinting).
Abnormal gene imprinting increases IGF2 (a growth signal). Constant growth signaling makes adrenal cortex cells divide too much, allowing cancer to form.Wnt/β-catenin pathway activation (CTNNB1).
This pathway tells cells to grow. Mutations that keep it “ON” push adrenal cells to multiply and ignore normal stop signals.ZNRF3 loss.
ZNRF3 is a natural brake on Wnt signaling. Losing it removes that brake and lets cells grow more easily into tumors.MEN1 gene changes.
MEN1 is another tumor-suppressor gene. When it is faulty, several endocrine organs, including the adrenal cortex, can form tumors, rarely carcinomas.Lynch syndrome (mismatch-repair defects).
When the DNA “spell-check” system is broken, mutations accumulate. Over time this can lead to cancers, including rare ACC.Carney complex (PRKAR1A defects).
Abnormal cell signaling in this syndrome can promote adrenal growths. Most are benign, but cancer risk is increased.Familial ACC without a known gene.
Some families have more ACC than expected. A yet-unknown inherited factor may raise risk in these families.Congenital adrenal hyperplasia (chronic ACTH drive).
Long-term stimulation of the adrenal cortex may cause overgrowth. Rarely, overgrown cells can take on cancerous changes.Chronic ACTH stimulation from pituitary disease.
Persistent ACTH can encourage cortex enlargement. It is not a common cause, but it may contribute in a few people.Childhood ionizing radiation exposure.
Radiation can damage DNA in growing tissues. Years later, this damage can promote cancer, including ACC.Endocrine-disrupting chemicals (certain pesticides/industrial compounds).
Some chemicals mimic hormones or disturb hormone pathways. Long exposure may raise risk by pushing abnormal adrenal growth.Chronic adrenal injury or inflammation.
Repeated cellular damage and healing can cause DNA errors. Over time, rare malignant transformation can occur.Genomic instability and telomere shortening.
When chromosomes become unstable, cells can gain cancer traits like limitless growth and invasion.Epigenetic changes (abnormal DNA methylation/histones).
These “software” errors in gene control can switch off tumor-suppressor genes or switch on growth genes.IGF2 overexpression (somatic).
ACC often shows very high IGF2 levels. IGF2 drives growth and survival signals in tumor cells.Chromosomal catastrophes (chromothripsis).
A single event can shatter and rejoin chromosomes incorrectly. This creates multiple cancer-helping changes at once.Mitochondrial dysfunction and excess ROS.
Energy factories inside cells may leak reactive molecules that damage DNA, nudging cells toward cancer.Rare progression of an adrenal adenoma.
Most adenomas stay benign. Very rarely, a benign tumor acquires more mutations and turns malignant.Biologic susceptibility (age extremes and female predominance).
ACC peaks in young children and adults (especially females). This pattern hints that hormone environment and development may influence risk.
Symptoms
Abdominal pain or pressure.
The tumor grows in a tight space and can press on nearby structures, causing dull or aching pain.A palpable abdominal mass or fullness.
A large adrenal tumor can be felt or make the belly look swollen or asymmetrical.Unexplained weight loss and poor appetite.
Fast-growing cancers use energy and may suppress appetite, leading to weight loss.Fatigue and muscle weakness.
Cancer-related inflammation and hormone imbalance (like too much cortisol or low potassium) cause tiredness and weak muscles.High blood pressure.
Excess aldosterone or cortisol raises blood pressure through salt and water retention and vessel effects.High blood sugar or new diabetes.
Too much cortisol acts like long-term stress, pushing blood sugar up and causing insulin resistance.Easy bruising, thin skin, slow healing, purple stretch marks.
Cortisol weakens skin and blood vessels, so bruises and striae (especially on the belly) appear easily.Round “moon” face, central weight gain, and limb muscle loss.
Cortisol changes fat and protein balance, causing a typical Cushing-like body shape.Mood changes: anxiety, irritability, depression, or poor sleep.
Hormone surges affect brain chemistry and daily rhythms.Frequent infections.
Cortisol suppresses immune defenses, so common infections may happen more often or last longer.Low potassium symptoms: cramps, weakness, palpitations.
Aldosterone makes the kidney waste potassium, which the muscles and heart need to work normally.Androgen excess in females: hirsutism, acne, deeper voice, irregular or absent periods.
Extra androgens from the tumor cause male-type features.Early puberty or rapid growth in children.
Sex steroid excess speeds bone maturation and secondary sexual features.Estrogen excess in males: breast enlargement and low libido; in females: unexpected uterine bleeding.
Too much estrogen shifts normal hormonal balance.Back pain, bone pain, or fractures.
Cortisol causes bone loss (osteoporosis), and cancer spread can also hurt bones.
Diagnostic tests
A) Physical exam
1) General inspection for hormone signs.
The doctor looks for cushingoid features (round face, purple striae), excess hair growth, acne, gynecomastia, or signs of early puberty. These visible clues suggest a functioning tumor.
2) Vital signs and body measures.
Blood pressure, heart rate, weight, height, and body mass index are checked. In children, growth charts show if growth is unusually fast or slowed, which can reflect hormone excess.
3) Abdominal examination.
Gentle palpation and percussion check for tenderness, a mass, or organ enlargement. A large adrenal tumor can sometimes be felt high in the flank or upper abdomen.
B) Manual tests
4) Manual blood pressure (including seated and standing).
Repeated, careful cuff measurements help confirm persistent hypertension and check for posture-related changes. This aids detection of cortisol or aldosterone excess.
5) Proximal muscle strength testing.
The doctor asks you to rise from a chair or lift your arms and legs against resistance. Cortisol excess often causes shoulder and hip girdle weakness.
6) Hirsutism scoring and waist measurement.
The modified Ferriman–Gallwey score grades excess hair growth in females. Waist circumference tracks central obesity linked to cortisol excess.
C) Lab and pathological tests
7) 1-mg overnight dexamethasone suppression test (DST).
You take dexamethasone at night; a healthy adrenal slows cortisol by morning. If morning cortisol stays high, it suggests autonomous cortisol production by a tumor.
8) 24-hour urinary free cortisol (UFC).
Collecting urine for 24 hours measures total cortisol output. Persistently high values support cortisol overproduction.
9) Late-night salivary cortisol.
Cortisol should be low late at night. A high bedtime saliva cortisol means the normal day-night rhythm is lost, pointing to Cushing physiology.
10) Plasma ACTH.
Low ACTH with high cortisol points to an adrenal source (the adrenal acts on its own). This helps separate adrenal Cushing from pituitary or ectopic ACTH causes.
11) DHEA-S and androgen panel (testosterone, androstenedione).
High adrenal androgens suggest a functioning adrenal tumor, especially in women and children with virilization signs.
12) Estradiol (and sex hormone profile).
In males, high estradiol with low testosterone can explain feminization. In females, abnormal estradiol may cause unexpected bleeding.
13) Aldosterone-renin ratio (with confirmatory saline test when needed).
A high ratio suggests autonomous aldosterone production (Conn physiology). Confirmation tests prove that aldosterone stays high even when the body should turn it down.
14) Basic metabolic panel (potassium, sodium, glucose) and HbA1c.
Low potassium and high sodium fit aldosterone excess; high glucose fits cortisol excess. These simple labs track severity and guide treatment.
15) Plasma or urine metanephrines (pheochromocytoma screen).
This is a safety step before any needle biopsy or surgery. It makes sure the tumor is not a medullary tumor (pheochromocytoma) that could trigger dangerous blood-pressure spikes.
16) Surgical pathology with Weiss score and Ki-67 index.
After tumor removal (or rarely core biopsy), the pathologist looks at the tissue. The Weiss score and the Ki-67 (a growth marker) help confirm carcinoma and estimate aggressiveness.
D) Electrodiagnostic tests
17) 12-lead electrocardiogram (ECG).
Low potassium can cause abnormal heart rhythms, and high blood pressure stresses the heart. ECG checks rhythm and conduction and looks for strain.
18) 24-hour Holter ECG or ambulatory blood pressure monitoring.
A day-long recording detects silent rhythm problems and confirms sustained hypertension, helping tailor treatment and anesthesia planning.
E) Imaging tests
19) Adrenal-protocol CT scan with contrast.
CT shows tumor size, shape, fat content, and local invasion. Features like large size, irregular borders, high density, and delayed contrast washout suggest carcinoma. The same scan helps check lymph nodes and nearby organs.
20) MRI of the adrenals (with chemical-shift sequences).
MRI distinguishes fat-rich benign adenomas from malignant lesions and shows invasion into vessels (like the inferior vena cava). PET-CT can be added in many centers to assess spread and active disease, but MRI alone already answers key questions about local behavior.
Non-pharmacological treatments
(15 Physiotherapy / activity & body care; Mind-Body & psychological support; “Gene therapy” is not an approved supportive method in ACC; instead, I include genetic counseling and learning about inherited risk when relevant.)
1) Pre-surgery breathing and walking plan (prehabilitation).
Purpose: prepare your heart and lungs for anesthesia and recovery.
Mechanism: gentle daily walking, incentive spirometry, and posture drills improve lung expansion, lower post-op complications, and speed discharge.
Benefits: better stamina, less pneumonia risk, easier coughing and deep breathing after surgery.
2) Post-surgery early mobilization.
Purpose: reduce blood clots and lung problems.
Mechanism: guided sitting up, standing, and short hallway walks within 24 hours if safe.
Benefits: faster bowel recovery, less muscle loss, improved mood.
3) Core and back strengthening.
Purpose: protect the surgical area and reduce pain.
Mechanism: physiotherapist-led activation of core muscles (transversus abdominis, multifidus), gentle hip and back mobility.
Benefits: better posture, less strain on the incision, easier daily tasks.
4) Pelvic and diaphragm release breathing.
Purpose: ease abdominal tightness.
Mechanism: slow diaphragmatic breaths and side-lying rib mobilization.
Benefits: lower pain and anxiety; supports cough and lymph flow.
5) Lymphedema prevention basics (if nodes removed).
Purpose: reduce swelling risk.
Mechanism: elevation, gentle massage, and compression as taught by therapist.
Benefits: comfort, better movement, fewer skin problems.
6) Balance and fall-prevention training.
Purpose: steroids and muscle loss increase fall risk.
Mechanism: tandem stance, step-ups, chair rises with support.
Benefits: safer walking, confidence.
7) Resistance training with light weights or bands.
Purpose: fight muscle loss from cortisol excess or chemotherapy.
Mechanism: 2–3 sessions/week with large muscle groups.
Benefits: strength, glucose control, energy.
8) Flexibility and gentle yoga (oncology-adapted).
Purpose: relieve stiffness, improve sleep.
Mechanism: slow stretches avoiding strain on incision.
Benefits: less pain, better mood.
9) Energy conservation and pacing.
Purpose: manage fatigue.
Mechanism: plan heavy tasks for the time of day you feel best; rest before you crash.
Benefits: steadier energy across the day.
10) Safe cardio (as cleared): walking or cycling.
Purpose: protect the heart (steroids can raise BP, chemos can stress the heart).
Mechanism: 20–30 minutes most days at easy pace.
Benefits: better blood pressure and mood.
11) Bone health exercises.
Purpose: cortisol excess weakens bones.
Mechanism: weight-bearing walks, sit-to-stands, heel drops.
Benefits: maintain bone density and balance.
12) Incision care education.
Purpose: prevent infection.
Mechanism: keep site clean/dry, hand hygiene, signs to watch for.
Benefits: safer healing.
13) Nutrition coaching for hypercortisolism.
Purpose: control weight, sugar, and salt.
Mechanism: higher protein, adequate calcium/vitamin D, limit sodium and ultra-processed foods; small, regular meals.
Benefits: steadier glucose, less swelling, stronger muscles and bones.
14) Salt and potassium guidance if hyperaldosteronism.
Purpose: manage blood pressure and electrolytes.
Mechanism: reduce added salt; potassium-rich foods (unless restricted).
Benefits: fewer cramps, better BP.
15) Sleep hygiene plan.
Purpose: steroid excess and stress disturb sleep.
Mechanism: fixed bedtime, dark cool room, screens off 1 hour before bed.
Benefits: better energy and healing.
16) Mindfulness-based stress reduction.
Purpose: reduce anxiety and pain.
Mechanism: breath focus, body scan, guided imagery 10–20 minutes daily.
Benefits: calmer mood, lower stress hormones.
17) Cognitive-behavioral therapy (CBT) for cancer-related distress.
Purpose: handle fear of recurrence and uncertainty.
Mechanism: skills to challenge worry thoughts, plan valued activities.
Benefits: less anxiety/depression; better treatment adherence.
18) Support group or peer mentor.
Purpose: share tips and reduce isolation in a rare cancer.
Mechanism: moderated groups in clinic or online.
Benefits: practical problem-solving and hope.
19) Pain self-management skills.
Purpose: complement pain meds.
Mechanism: heat/ice (as approved), relaxation, pacing, comfortable positioning.
Benefits: less pain flare, more control.
20) Nausea coping routines.
Purpose: help alongside anti-nausea drugs.
Mechanism: small bland meals, ginger tea, fresh air, acupressure at P6 point if taught.
Benefits: fewer vomiting episodes.
21) Sexual health and body-image counseling.
Purpose: hormone changes and scars affect intimacy.
Mechanism: counseling with trained clinician; pelvic PT if needed.
Benefits: better communication and comfort.
22) Work and school accommodations.
Purpose: protect energy during treatment.
Mechanism: lighter duties, flexible hours, rest breaks.
Benefits: maintain role and income.
23) Financial navigation and social work help.
Purpose: lower stress from costs and travel.
Mechanism: connect with insurance, charity rides, lodging programs.
Benefits: better access to care.
24) Genetic counseling (when family risk suspected).
Purpose: check for inherited syndromes (e.g., Li-Fraumeni, Lynch).
Mechanism: review family history; consider testing.
Benefits: guides screening for relatives and follow-up plans. Cancer.gov
25) Clinical-trial education and eligibility review.
Purpose: access new therapies.
Mechanism: discuss open trials for systemic therapy or immunotherapy.
Benefits: more options in advanced ACC. Cancer.govClinicalTrials.gov
Drug treatments
Doses and schedules vary by body size, kidney/liver function, drug levels, and combinations. Always individualize with your oncology/endocrine team.
1) Mitotane (o,p′-DDD).
Class: adrenolytic, anti-steroidogenesis.
Typical use: cornerstone in advanced disease; sometimes after surgery in higher-risk cases.
Dose/time: started low and increased gradually; given continuously; blood levels are monitored (commonly a target range is used in practice).
Purpose: kills adrenal tumor cells and blocks cortisol production.
Mechanism: selective adrenal cortex toxicity; CYP induction affects many drugs; patients often need higher steroid replacement.
Main side effects: nausea, diarrhea, fatigue, confusion, neuropathy; strong drug interactions; adrenal insufficiency if not supplemented. PubMedOxford AcademicScienceDirect
2) EDP-Mitotane regimen (etoposide + doxorubicin + cisplatin + continuous mitotane).
Class: combination cytotoxic chemotherapy plus adrenolytic.
Dose/time: cyclical IV chemo (e.g., every 3–4 weeks) with continuous oral mitotane; exact milligram doses depend on protocol.
Purpose: first-line therapy for advanced/metastatic ACC in many centers.
Evidence: improved response rate and progression-free survival vs streptozocin-mitotane in the FIRM-ACT trial.
Side effects: low blood counts, infection risk, hair loss, nausea, kidney injury (cisplatin), heart risks (doxorubicin), neuropathy, fatigue. New England Journal of MedicinePubMedEndokrinologie
3) Streptozocin + mitotane.
Class: alkylating agent + adrenolytic.
Use: alternative to EDP-M when EDP-M is not suitable.
Evidence: lower response rate than EDP-M but used in selected patients.
Side effects: kidney toxicity, nausea, low counts. PubMed
4) Carboplatin- or cisplatin-based doublets (with etoposide, paclitaxel, or gemcitabine).
Use: second-line or if EDP-M is not tolerated.
Purpose/Mechanism: DNA damage and microtubule disruption to slow tumor growth.
Side effects: low counts, fatigue, neuropathy; kidney and nerve issues with platinums. Cancer.gov
5) Gemcitabine + capecitabine (sometimes with mitotane).
Use: later-line for metastatic ACC in small series.
Side effects: hand-foot redness, diarrhea, low counts, fatigue. Cancer.gov
6) Temozolomide (± mitotane).
Class: alkylating.
Use: small studies; can be considered in refractory settings.
Side effects: low counts, fatigue, nausea. Cancer.gov
7) Pembrolizumab (PD-1 inhibitor).
Class: immunotherapy checkpoint inhibitor.
Use: selected advanced ACC; response rates modest overall but some durable responders.
Side effects: immune-related inflammation (thyroid, liver, lung, colon), fatigue.
Evidence: phase II studies show objective response in a minority of patients. PubMed+1
8) Avelumab (PD-L1 inhibitor).
Use: studied in metastatic ACC; low response rate overall; sometimes given with ongoing mitotane.
Side effects: immune-related effects; infusion reactions. jitc.biomedcentral.com
9) Metyrapone.
Class: steroidogenesis inhibitor.
Use: quickly lowers cortisol when the tumor causes Cushing’s syndrome; used as bridging therapy before surgery or while starting mitotane.
Side effects: high blood pressure and low potassium can worsen; dizziness, GI upset. Cancer.gov
10) Ketoconazole (at higher endocrine doses).
Class: antifungal with steroid-blocking action.
Use: lowers cortisol over days to weeks.
Side effects: liver toxicity risk, drug interactions (CYP3A4). Cancer.gov
11) Osilodrostat.
Class: 11β-hydroxylase inhibitor.
Use: off-label in some centers for rapid cortisol control.
Side effects: low cortisol, GI upset, fatigue; monitoring needed. Cancer.gov
12) Mifepristone.
Class: glucocorticoid receptor blocker.
Use: treats Cushing’s symptoms when cortisol cannot be lowered enough.
Side effects: low potassium, uterine bleeding, fatigue; cannot use cortisol blood tests to monitor (use symptoms). Cancer.gov
13) Spironolactone / Eplerenone.
Class: mineralocorticoid receptor blockers.
Use: control blood pressure and potassium when aldosterone is high.
Side effects: high potassium, dizziness; spironolactone can cause breast tenderness. Cancer.gov
14) Glucocorticoid and sometimes mineralocorticoid replacement (e.g., hydrocortisone ± fludrocortisone) during mitotane therapy or after adrenal removal.
Use: prevent adrenal insufficiency because mitotane increases steroid breakdown and bilateral adrenal loss removes hormone production.
Notes: doses are often higher than usual because mitotane speeds up steroid metabolism. ScienceDirect
15) Standard supportive medicines (anti-nausea, antibiotics when needed, bone-protective agents if osteoporosis, diabetes and blood-pressure medicines).
Use: treat side effects from hormones and chemotherapy.
Benefit: keeps treatment on track and protects quality of life. Cancer.gov
Dietary “molecular” supplements
Supplements can interact with mitotane and chemotherapies. Always clear with your doctors and pharmacists first.
1) Vitamin D (if low). Helps bone strength when cortisol is high. Typical adult replacement follows blood levels. Do not exceed without testing.
Mechanism: supports calcium absorption and bone remodeling.
2) Calcium (diet first; pill only if prescribed). Strengthens bones; can be paired with vitamin D.
Mechanism: mineral for bone matrix; check for kidney stones risk.
3) Omega-3 (fish oil) from food or capsules if approved. May help appetite and weight maintenance in some cancer settings.
Mechanism: anti-inflammatory lipid mediators; may support muscle.
4) Ginger (tea/capsules). Can reduce nausea along with anti-emetic drugs.
Mechanism: acts on gut receptors that influence nausea.
5) Probiotics (specific strains) for diarrhea prevention during some treatments—only if your team says it’s safe.
Mechanism: supports gut microbiome; avoid if severely immunosuppressed.
6) Soluble fiber (oats, psyllium). Smooths bowel movements with diarrhea or constipation.
Mechanism: water-holding gel in intestine.
7) Whey or plant protein supplements if intake is low.
Mechanism: provides amino acids to fight muscle loss from cortisol or chemo.
8) Magnesium (food first; supplement only if low).
Mechanism: supports muscles and heart rhythm; losses can occur with some medicines.
9) B-complex (only if deficient).
Mechanism: supports energy metabolism; avoid mega-doses.
10) Multivitamin at standard dose if diet is poor.
Mechanism: fills small gaps without high doses that might interact.
(Evidence for supplements in ACC itself is limited; the goal is symptom and nutrition support. Discuss every product with your team, because mitotane and many chemo drugs have major interactions.) Cancer.gov
Immunity booster / regenerative / stem-cell” drugs —
There are no approved stem-cell drugs to cure ACC. What can truly help are evidence-based immune-supportive measures and hematologic growth factors used by oncology teams:
1) G-CSF (filgrastim/pegfilgrastim).
Use: prevents or treats low white cells from chemotherapy to reduce infection risk.
Mechanism: stimulates bone marrow to make neutrophils.
Function: lowers febrile neutropenia risk; timing is set by the chemo cycle.
2) Erythropoiesis-stimulating agents (ESAs) in selected patients.
Use: treat chemo-related anemia when transfusion alternatives are considered appropriate.
Mechanism: stimulates red-cell production.
Function: reduces transfusions; used under strict rules.
3) Vaccinations (influenza, COVID-19, pneumococcal) per oncology guidance.
Function: lowers severe infection risk.
4) Immunotherapy (pembrolizumab/avelumab) in selected advanced cases.
Function: removes immune “brakes” so T-cells can attack cancer; only a minority respond. PubMed+1
5) Nutrition-and-exercise “immune fitness.”
Function: adequate protein, sleep, and activity help immune recovery after chemo.
6) Infection-prevention bundles.
Function: hand hygiene, central-line care, dental care, and prompt fever reporting.
(Avoid any clinic or website offering “stem-cell cures” for ACC—these are unproven and may be dangerous.)
Surgeries
1) Open adrenalectomy with en-bloc resection.
What: remove the adrenal tumor through an open incision; remove attached tissues if invaded.
Why: best chance for complete removal (R0), which gives the highest chance for long-term control. Minimally invasive approaches are usually avoided for suspected ACC because of tumor rupture risk. Cancer.govPubMed
2) Lymph-node dissection (regional).
What: remove nearby nodes at risk.
Why: staging accuracy and possible local control in selected cases. PubMed
3) Inferior vena cava (IVC) thrombectomy (if tumor thrombus).
What: remove tumor extension into the big vein.
Why: restore blood flow and allow complete tumor resection. Cancer.gov
4) Metastasectomy (e.g., lung or liver) in selected patients.
What: remove limited metastases if feasible.
Why: symptom relief and potential survival benefit in highly selected cases. Cancer.gov
5) Palliative debulking or bypass procedures.
What: reduce bulky disease when cure is not possible.
Why: relieve pain, bleeding, or obstruction; improve quality of life. Cancer.gov
Preventions
There is no sure way to prevent ACC, but the steps below may reduce harm or catch problems early:
Know family history; seek genetic counseling if relatives had ACC or known syndromes (Li-Fraumeni, Lynch). Cancer.gov
Do regular health checks for blood pressure, glucose, and weight changes.
See a doctor promptly for unexplained rapid weight gain, easy bruising, severe acne or hair growth, new high blood pressure, or low potassium.
Keep a healthy body weight and stay active to protect the heart and bones, especially if hormones are high.
Do not smoke; smoking worsens surgical and heart risks.
Limit alcohol to lower liver stress during treatment.
Keep vaccines up to date to lower infection risk during therapy.
Use a medication wallet card listing mitotane or other drugs and interactions.
Bone health habits (calcium/vitamin D food sources; weight-bearing exercise).
Participate in recommended imaging and lab follow-up after surgery to catch recurrence early. PubMed
When to see doctors
Call urgently or go to emergency if you have fever ≥38°C, chest pain, shortness of breath, severe belly pain, confusion, sudden weakness, or signs of adrenal crisis (severe vomiting, low blood pressure, fainting).
Contact your oncology/endocrine team within 24 hours for vomiting that prevents fluids, rapid swelling of legs, yellow skin/eyes, new severe headaches, or blood sugar very high or very low.
Routine follow-up: keep every planned visit for lab tests (hormones, electrolytes), mitotane levels if used, and scheduled scans. Cancer.gov
What to eat and what to avoid
What to eat: small regular meals with lean protein (fish, poultry, eggs, beans), high-fiber carbs (whole grains, oats), colorful vegetables and fruits, and dairy or fortified alternatives for calcium and vitamin D. Choose potassium-rich foods (bananas, oranges, tomatoes, spinach) if your clinician says your potassium is low. Drink enough water.
What to limit/avoid: excess salt (especially if aldosterone is high), sugary drinks (to help glucose control), ultra-processed foods, and deep-fried items. Alcohol should be minimal. Herbal supplements with strong CYP interactions (like St. John’s wort) or grapefruit can affect drug levels; ask your team first, especially if you take mitotane or chemo. Cancer.gov
Frequently Asked Questions (FAQs)
1) Is surgery really the most important treatment?
Yes. If the tumor can be safely removed completely, open adrenalectomy offers the best chance for long-term control. Cancer.govPubMed
2) Why do doctors talk so much about mitotane?
Mitotane specifically targets adrenal tissue and is central in advanced ACC and sometimes after surgery in higher-risk cases. Blood levels are monitored to balance benefit and side effects. PubMedOxford Academic
3) What is the standard chemo?
Many centers use EDP-Mitotane (etoposide, doxorubicin, cisplatin + mitotane) first-line for advanced disease because it improved response rate and time without progression compared with streptozocin-mitotane. New England Journal of MedicinePubMed
4) Does chemo cure ACC?
It can shrink or slow cancer, but cure is uncommon in metastatic disease. Surgery offers the best chance for cure when possible. Cancer.gov
5) Does immunotherapy work?
Some patients respond to pembrolizumab or avelumab, but overall response rates are modest. Trials are ongoing. PubMed+1jitc.biomedcentral.com
6) Why do I need steroid pills after surgery or on mitotane?
Because either the gland is removed or mitotane speeds up steroid breakdown. Replacement prevents adrenal crisis. ScienceDirect
7) How often will I need scans after surgery?
Follow-up imaging is scheduled regularly (often every few months at first) to catch recurrence early; your team will set the exact plan. PubMed
8) What if my blood pressure and sugars are high?
Hormone excess can cause this. Doctors treat with targeted hormone-blocking drugs and standard BP and diabetes medicines to protect your organs. Cancer.gov
9) Are there foods that shrink the tumor?
No foods cure ACC. Nutrition supports strength and helps treatment side effects. Avoid interactions (e.g., grapefruit) with certain drugs. Cancer.gov
10) Should I enroll in a clinical trial?
If you are eligible, trials may offer access to new options, especially for advanced disease. Discuss with your team. Cancer.gov
11) What if surgery cannot remove all the tumor?
Options include systemic therapy, palliative surgery or radiation for symptoms, and trials. Cancer.gov
12) Is radiation useful?
ACC is relatively radio-resistant, but radiation can be used to relieve pain or control a spot of disease. Cancer.gov
13) Can children get ACC?
Yes, but it is different in many ways; specialized pediatric centers follow separate guidance. NCBI
14) How rare is ACC?
It is rare; many people benefit from care at centers with experience in adrenal cancers and multidisciplinary teams. PubMed
15) What is the outlook?
Outcomes vary by stage, whether surgery can fully remove the tumor, and how it responds to therapy. Close follow-up is essential. Cancer.gov
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: September 08, 2025.
