Adrenal cortex adenocarcinoma, often called adrenocortical carcinoma (ACC), is a rare and aggressive cancer that starts in the outer layer (cortex) of an adrenal gland. Each person has two adrenal glands that sit on top of the kidneys. The adrenal cortex makes important hormones such as cortisol, aldosterone, and androgens (sex hormones). In ACC, abnormal cells grow in this layer and form a tumor. The tumor can be functional (it makes extra hormones) or non-functional (it does not make hormones). Functional tumors may cause clear hormone-related symptoms like weight gain, high blood pressure, or hair growth. Non-functional tumors often grow silently until they are large. ACC can spread to nearby tissues and to distant organs like the liver, lungs, or bones. Early surgery gives the best chance of cure. Many people also need medicines, radiation, or other treatments to control the disease or to control extra hormone production. Lifelong follow-up is important.
Adrenal cortex adenocarcinoma—more commonly called adrenocortical carcinoma (ACC)—is a rare, aggressive cancer that starts in the outer layer (cortex) of the adrenal gland. The cortex makes steroid hormones that control salt balance, blood pressure, stress response, sex traits, and growth. When a tumor forms, it can grow locally and spread to other organs. Many tumors also overproduce hormones, which causes body-wide changes such as weight gain, high blood pressure, or changes in hair and sex features. Some tumors do not make extra hormones and only show symptoms when they get large. Diagnosis needs careful hormone testing and imaging. Treatment is usually surgery, sometimes with medicines like mitotane, chemotherapy, and radiation. Early detection and complete surgical removal give the best chance for long-term control.
Other names
Adrenal cortex adenocarcinoma is most often referred to as adrenocortical carcinoma (ACC). Other terms you may see include adrenal cortical carcinoma, malignant adrenal cortical tumor, and functional adrenal cortical carcinoma when it makes excess hormones. When the tumor does not make hormones, it may be called non-functional ACC. Pathology reports may use words like oncocytic adrenocortical carcinoma, myxoid adrenocortical carcinoma, or sarcomatoid adrenocortical carcinoma to describe special microscopic patterns. In staging systems, you may also see ENSAT stage I–IV ACC. These names all point to the same disease: a malignant tumor arising from the steroid-producing cells of the adrenal cortex.
Types
By hormone behavior
Cortisol-secreting ACC: causes Cushing-like features (weight gain in the trunk, round face, diabetes, muscle weakness, bruising).
Androgen-secreting ACC: causes virilization (acne, excess facial/body hair, deepening voice, menstrual changes).
Estrogen-secreting ACC: in men causes breast enlargement and low libido; in women causes uterine bleeding or feminizing changes.
Aldosterone-secreting ACC: causes resistant high blood pressure and low potassium.
Mixed-secreting ACC: more than one hormone pathway is overactive.
Non-functional ACC: no hormone excess; symptoms come from mass effect.
By microscopic pattern (pathology)
Conventional (usual) ACC
Oncocytic ACC (cells packed with mitochondria)
Myxoid ACC (gel-like stroma)
Sarcomatoid ACC (rare, very aggressive)
By stage (ENSAT)
Stage I–II: tumor limited to the adrenal, smaller or larger than 5 cm.
Stage III: local invasion or positive lymph nodes.
Stage IV: distant spread (liver, lung, bone, etc.).
By clinical setting
Sporadic (most cases)
Hereditary syndromic ACC (linked to certain gene conditions; often pediatric or early adult onset)
Causes and risk factors
Most ACCs are sporadic (no clear cause). Some are linked to inherited gene changes or long-term growth-signaling problems in adrenal cells.
Li-Fraumeni syndrome (TP53 mutation). A strong, well-known genetic cause. Faulty p53 “guardian” protein allows damaged adrenal cells to become cancer.
Beckwith–Wiedemann spectrum / 11p15 imprinting defects. Overgrowth signals (often IGF2 overexpression) push adrenal cells to divide too much, raising cancer risk in children.
Heritable TP53 R337H variant (Brazilian founder). A regional TP53 change that specifically increases pediatric and young adult ACC risk.
Multiple Endocrine Neoplasia type 1 (MEN1). MEN1 pathway disruption can promote adrenal cortical tumors, rarely malignant.
Lynch syndrome (DNA mismatch-repair defects). Weaker link than colorectal/uterine cancer, but repair failure may permit adrenal cancers to arise.
Carney complex (PRKAR1A). Abnormal cAMP signaling can drive adrenal nodules; rarely progresses to ACC.
DICER1 syndrome. Aberrant microRNA processing can deregulate growth genes in adrenal cells.
β-catenin/CTNNB1 pathway activation. A common somatic tumor mutation; keeps growth signals “on,” aiding malignant change.
IGF2 overexpression (somatic). Very frequent in ACC; acts like a “grow” command for adrenal cells.
Chromosomal instability. Gains/losses of DNA segments disrupt many control genes at once, allowing malignant behavior.
Congenital adrenal hyperplasia (chronic ACTH drive). Long-term stimulation may cause hyperplasia and nodules; rarely malignant, but risk is higher than average.
Prior abdominal or flank radiation in childhood. Radiation can damage DNA in adrenal cells and slightly increase later cancer risk.
Family history of adrenal tumors. May point to shared genes or modifiers not yet fully identified.
Very young age (childhood) with overgrowth features. Reflects underlying imprinting/growth pathway disorders.
Long-standing adrenal cortical adenoma with atypia. Rarely, a benign adenoma acquires new mutations and transforms into carcinoma.
Endocrine-disrupting exposures (possible). Some pesticides/industrial chemicals may alter steroid pathways—evidence is limited and not definitive.
Immune surveillance defects. If the immune system fails to remove abnormal adrenal cells, cancers can escape—conceptual, not a single cause.
Chronic inflammation/oxidative stress in adrenal tissue. Persistent cell injury may promote DNA damage over time.
Metabolic stress (obesity/insulin excess). High insulin/IGF signaling might encourage adrenal growth; association is biologically plausible but modest.
Unknown factors. In most adults, no clear driver is found; multiple small hits to growth control probably accumulate.
Symptoms and signs
Abdominal or flank pain. The growing mass stretches tissues or presses on nearby organs.
A feeling of fullness or a lump. Large tumors push outward; some people feel or their doctor detects a mass.
Unplanned weight loss and fatigue. A sign of advanced cancer in many organs, including adrenal.
New or resistant high blood pressure. From cortisol or aldosterone excess, or from mass effect on vessels.
Low potassium symptoms (muscle cramps, weakness, palpitations). Typical for aldosterone-secreting tumors.
Cushing-like changes (central weight gain, round face, thin skin, purple stretch marks, easy bruising). Due to cortisol excess.
New-onset diabetes or poor glucose control. Cortisol raises blood sugar.
Muscle weakness, especially in thighs/hips. Cortisol breaks down muscle proteins, causing proximal myopathy.
Excess body or facial hair, acne, deepening voice, irregular or absent periods. Androgen excess effects in women.
Early puberty in children. Sex steroids from the tumor speed secondary sex changes.
Breast enlargement and low libido in men. Estrogen-secreting tumors feminize.
Mood and sleep changes (anxiety, irritability, depression, insomnia). Cortisol affects brain and sleep cycles.
Bone thinning and fractures. Long-term cortisol excess leads to osteoporosis.
Swelling of legs (pitting edema). Hormone-driven fluid shifts or vena cava compression by a large tumor.
Symptoms from spread (cough, shortness of breath, bone pain, yellow eyes/skin). Reflects metastasis to lung, bone, or liver.
Diagnostic tests
Below are grouped tests with plain explanations. Doctors usually combine hormone testing and imaging; they also rule out pheochromocytoma before any biopsy.
Physical examination
1) Cushingoid feature check.
Your doctor looks for round face, fat above the collarbone, thin skin, purple stretch marks, acne, and easy bruising. These visible clues suggest cortisol excess from a functioning tumor.
2) Abdominal palpation.
Gentle pressing on the belly and flank can reveal a firm, deep mass in the adrenal region or tenderness from a large tumor.
3) Blood pressure measurement.
One or more readings help screen for hypertension from cortisol or aldosterone excess. Very high or difficult-to-control values raise suspicion.
4) Hair/skin and sexual characteristic assessment.
Clinicians look for new facial/body hair, acne, scalp hair thinning, virilization in women, or breast enlargement in men—clues to androgen or estrogen excess.
“Manual” bedside tests
5) Orthostatic blood pressure.
Pressure and pulse are checked lying and then standing. Hormone-driven volume changes or severe cortisol effects can blunt normal responses.
6) Manual muscle strength testing.
Doctors test hip and shoulder muscles against resistance. Weakness out of proportion to effort suggests cortisol-related myopathy.
7) Pitting-edema assessment.
Pressing a finger over the shin or ankle checks for a dent that persists, a sign of fluid overload from hormones or venous compression.
Laboratory and pathological tests
8) Overnight 1-mg dexamethasone suppression test (DST).
You take dexamethasone at night; morning cortisol is measured. Normal adrenal glands “quiet down” and cortisol falls. Failure to suppress points to autonomous cortisol production.
9) 24-hour urinary free cortisol (UFC).
You collect urine for a day. Persistently high cortisol confirms overproduction and helps grade severity.
10) Late-night salivary cortisol.
Saliva collected at bedtime shows if cortisol stays abnormally high when it should be low—useful for detecting mild hypercortisolism.
11) Plasma ACTH.
Low ACTH with high cortisol suggests the adrenal is the source (ACTH-independent Cushing’s). Normal/high ACTH suggests a pituitary or ectopic source instead.
12) Androgen/estrogen panel (DHEA-S, androstenedione, testosterone, estradiol).
High DHEA-S and related androgens indicate a cortical source; estradiol may be elevated in feminizing tumors.
13) Aldosterone-to-renin ratio plus electrolytes (potassium/sodium).
A high ratio with low potassium fits aldosterone excess. This pattern can be seen in aldosterone-secreting ACC.
14) Histopathology with Weiss score, Ki-67, and immunostains (SF-1, inhibin, Melan-A).
If tissue is obtained (usually after adrenalectomy; biopsy is avoided unless necessary and only after ruling out pheochromocytoma), the pathologist uses the Weiss criteria and Ki-67 index to judge malignancy and aggressiveness. SF-1 confirms adrenal cortical origin.
Electrodiagnostic tests
15) Electrocardiogram (ECG).
Checks heart rhythm. Low potassium from aldosterone excess can cause arrhythmias or characteristic ECG changes; severe hypertension can strain the heart.
16) 24-hour Holter ECG monitoring.
Captures intermittent rhythm problems—palpitations or silent arrhythmias related to electrolyte shifts or blood pressure swings.
Imaging tests
17) Adrenal-protocol contrast-enhanced CT scan.
Core imaging test. It measures size, shape, density, and contrast washout. ACCs are often large, irregular, and have low washout—features suggesting malignancy.
18) MRI with chemical-shift imaging.
Helpful when CT is indeterminate. It looks for lipid content and invasion into veins or nearby organs. MRI is excellent for defining local spread.
19) FDG PET/CT (metabolic imaging).
Cancer cells consume more glucose, so they “light up” on PET. PET/CT helps stage disease, detect small metastases, and assess treatment response.
20) Abdominal ultrasound (and targeted ultrasound).
Not as sensitive as CT/MRI for the adrenal, but it can detect large masses and guide safe needle placement only when biopsy is indicated and pheochromocytoma has been excluded.
Non-pharmacological treatments
Important: Non-drug measures support, but do not replace, surgery or cancer medicines. Always coordinate with your oncology team.
Physiotherapy-focused
Early postoperative breathing exercises
Description: Simple deep-breathing and incentive spirometry after adrenal surgery.
Purpose: Prevent lung complications and improve oxygenation.
Mechanism: Expands small air sacs, clears secretions, improves lung mechanics.
Benefits: Less pneumonia, faster recovery, better energy.Graded walking program
Description: Daily step goals that slowly increase.
Purpose: Reduce fatigue, aid circulation, and maintain heart health.
Mechanism: Gentle aerobic load boosts mitochondria and blood flow.
Benefits: Better stamina, mood, and sleep; fewer clots.Light resistance training
Description: Bands or light weights 2–3 times/week under guidance.
Purpose: Combat muscle loss from cortisol excess and inactivity.
Mechanism: Stimulates muscle protein synthesis and neuromuscular function.
Benefits: Strength, balance, daily independence.Core and postural therapy
Description: Targeted core activation, spinal alignment, and posture drills.
Purpose: Reduce back pain from mass effect or deconditioning.
Mechanism: Re-educates stabilizers, improves load distribution.
Benefits: Less pain, better mobility and confidence.Flexibility and gentle yoga
Description: Short, low-intensity stretching routines.
Purpose: Maintain range of motion and reduce stiffness.
Mechanism: Lengthens soft tissue, down-regulates pain pathways.
Benefits: Comfort, ease of movement, calmer mind.Balance and fall-prevention training
Description: Static and dynamic balance tasks; home safety review.
Purpose: Reduce fracture risk with steroid-related bone loss.
Mechanism: Improves proprioception and reflexes.
Benefits: Fewer falls, greater independence.Scar and abdominal wall mobilization (post-op)
Description: Guided gentle massage and myofascial release after healing starts.
Purpose: Reduce adhesions and pain.
Mechanism: Improves tissue glide and circulation.
Benefits: Better posture, movement, and comfort.Pelvic diaphragm and breathing-posture synergy
Description: Coordinated diaphragmatic breathing with pelvic floor relaxation.
Purpose: Ease abdominal pressure and improve core function.
Mechanism: Normalizes pressure in trunk muscles.
Benefits: Less strain, smoother mobility.Fatigue management (“energy conservation”)
Description: Pacing, task grouping, rest scheduling.
Purpose: Make energy last through the day during treatment.
Mechanism: Balances activity/rest to avoid “crash cycles.”
Benefits: More control, less burnout.Lymphedema-aware limb care
Description: Gentle compression, elevation, and movement if nodes are removed.
Purpose: Prevent swelling after lymphadenectomy.
Mechanism: Supports lymph flow.
Benefits: Comfort, lower infection risk.Inspiratory muscle training
Description: Handheld device to strengthen breathing muscles.
Purpose: Improve exercise tolerance.
Mechanism: Progressive load on respiratory muscles.
Benefits: Less breathlessness, higher activity capacity.Pain-neuroscience education with movement
Description: Learn how pain works; pair with safe motion.
Purpose: Reduce fear and guarding.
Mechanism: Reframes pain signals; graded exposure.
Benefits: More movement, less disability.Neuromuscular electrical stimulation (selected cases)
Description: Low-level stimulation to weak muscles if exercise is limited.
Purpose: Maintain muscle mass during immobility.
Mechanism: Triggers contractions via motor nerves.
Benefits: Preserves strength, aids rehab start.Return-to-work conditioning
Description: Task-specific strengthening and endurance plans.
Purpose: Smooth and safe return to job roles.
Mechanism: Progressive overload matching job demands.
Benefits: Confidence, fewer setbacks.Bone-health exercise set
Description: Weight-bearing walks + safe impact drills + posture sets.
Purpose: Limit steroid-related bone loss.
Mechanism: Mechanical loading stimulates bone.
Benefits: Higher bone density, fewer fractures.
Mind-Body / Educational / Supportive
Mindfulness-based stress reduction
Description: Short daily attention and breathing practices.
Purpose: Lower anxiety, improve sleep and pain tolerance.
Mechanism: Calms stress circuits and HPA axis.
Benefits: Better mood and adherence to care.Cognitive behavioral therapy (CBT)
Description: Brief, structured sessions to reframe unhelpful thoughts.
Purpose: Manage fear of recurrence, body-image change, and fatigue.
Mechanism: Changes thought–feeling–behavior loops.
Benefits: Less distress, better coping.Sleep hygiene coaching
Description: Consistent schedule, light control, wind-down routine.
Purpose: Fix cortisol-related insomnia.
Mechanism: Resets circadian cues.
Benefits: Deeper sleep, sharper daytime focus.Nutrition counseling (oncology-informed)
Description: Adequate protein, balanced carbs, fiber, and hydration.
Purpose: Maintain weight and muscle; manage glucose and potassium.
Mechanism: Stabilizes blood sugar and electrolytes.
Benefits: Energy, fewer hospital visits.Genetic counseling and family risk guidance
Description: Review personal/family history and consider testing (e.g., TP53).
Purpose: Clarify risk and surveillance for relatives.
Mechanism: Evidence-based risk assessment.
Benefits: Early detection and informed decisions.Medication education for hormone control
Description: Teach how and when to take mitotane and steroid replacements.
Purpose: Improve safety and effectiveness.
Mechanism: Boosts adherence and early side-effect reporting.
Benefits: Fewer crises and ER visits.Guided imagery / music therapy
Description: Structured imagery or music to relax.
Purpose: Reduce procedural anxiety and pain.
Mechanism: Shifts attention, activates calming pathways.
Benefits: Comfort and sense of control.Financial and social-work navigation
Description: Help with insurance, leave from work, and transport.
Purpose: Reduce practical stress that worsens outcomes.
Mechanism: Removes barriers to care.
Benefits: Better continuity and adherence.Peer support or survivorship groups
Description: Share experiences and tips.
Purpose: Reduce isolation and fear.
Mechanism: Social modeling and reassurance.
Benefits: Hope and useful problem-solving.Advance-care planning conversation
Description: Discuss values, goals, and preferences early.
Purpose: Align care with what matters to you.
Mechanism: Shared decision-making.
Benefits: Clarity, dignity, and less stress.
Drug treatments
Safety first: Exact drug choice and dose depend on your body size, kidney/liver function, tumor stage, and other medicines. The following is educational and not a prescription.
Mitotane
Class: Adrenolytic agent.
Typical dose/time: Titrated to 2–6 g/day in divided doses; target blood levels (e.g., 14–20 μg/mL) with regular monitoring.
Purpose: Main systemic drug for ACC; also lowers cortisol production.
Mechanism: Toxic to adrenal cortical cells; alters steroid synthesis.
Side effects: Nausea, diarrhea, fatigue, neuro-cognitive effects; requires steroid replacement and drug-level monitoring.EDP-M regimen (Etoposide + Doxorubicin + Cisplatin + Mitotane)
Class: Combination cytotoxic chemotherapy plus adrenolytic.
Dose/time: Cycled every 3–4 weeks; mitotane is continued.
Purpose: For advanced or recurrent ACC to shrink or slow tumors.
Mechanism: Damages DNA and cell division; mitotane targets adrenal cells.
Side effects: Low blood counts, nausea, kidney/heart risks (monitor closely).Streptozocin (selected cases)
Class: Alkylating-like chemotherapy.
Dose/time: IV on schedules per protocol, often with mitotane.
Purpose: Alternative cytotoxic option when EDP-M is not suitable.
Mechanism: DNA damage in dividing cells.
Side effects: Nausea, kidney effects, low counts.Pembrolizumab
Class: Immune checkpoint inhibitor (PD-1).
Dose/time: IV every 3–6 weeks.
Purpose: For some advanced ACCs, especially after prior therapy.
Mechanism: Reactivates T-cells to attack cancer.
Side effects: Immune-related inflammation (thyroid, liver, lung, gut); needs prompt reporting.Nivolumab ± Ipilimumab
Class: PD-1 (± CTLA-4) checkpoint blockade.
Dose/time: IV every 2–4 weeks per protocol.
Purpose: Option in refractory ACC at experienced centers.
Mechanism: Enhances antitumor immune response.
Side effects: Immune-related toxicities; close monitoring.Cabozantinib (selected off-label contexts)
Class: Multi-target tyrosine kinase inhibitor (VEGFR/MET/AXL).
Dose/time: Oral daily (e.g., 60 mg, adjusted).
Purpose: Considered in select refractory cases.
Mechanism: Inhibits tumor angiogenesis and signaling.
Side effects: Fatigue, diarrhea, hand-foot syndrome, hypertension.Spironolactone
Class: Mineralocorticoid receptor antagonist.
Dose/time: 25–100 mg 1–2×/day (titrate).
Purpose: Control blood pressure and potassium with aldosterone excess.
Mechanism: Blocks aldosterone effects in kidneys.
Side effects: High potassium, breast tenderness; interacts with other drugs.Eplerenone
Class: Selective mineralocorticoid antagonist.
Dose/time: 25–50 mg 1–2×/day.
Purpose: Alternative to spironolactone with fewer sex-hormone side effects.
Mechanism: Blocks aldosterone receptor.
Side effects: High potassium; monitor labs.Ketoconazole
Class: Steroidogenesis inhibitor (also antifungal).
Dose/time: 200–400 mg 2–3×/day.
Purpose: Rapid cortisol control in Cushing’s from ACC.
Mechanism: Inhibits adrenal enzymes (e.g., CYP17A1).
Side effects: Liver toxicity, GI upset; monitor liver tests and drug interactions.Metyrapone
Class: Steroidogenesis inhibitor.
Dose/time: 250–1000 mg 2–4×/day.
Purpose: Another option to lower cortisol fast.
Mechanism: Blocks 11-β-hydroxylase.
Side effects: High androgens, high blood pressure, low potassium.Osilodrostat
Class: 11-β-hydroxylase inhibitor.
Dose/time: Start low and titrate; oral twice daily.
Purpose: Control cortisol when surgery or mitotane titration is pending.
Mechanism: Reduces cortisol synthesis.
Side effects: Low cortisol (adrenal insufficiency), QT prolongation; close monitoring.Mifepristone
Class: Glucocorticoid receptor antagonist.
Dose/time: Oral daily; dosing individualized.
Purpose: Treats hypercortisolism’s effects when enzyme blockers are not tolerated.
Mechanism: Blocks cortisol action at its receptor.
Side effects: Low potassium, endometrial thickening; requires careful follow-up.Antihypertensives (ACE inhibitors/ARBs, calcium-channel blockers)
Class: Blood pressure medications.
Dose/time: Standard dosing per guidelines.
Purpose: Control high blood pressure from hormones or pain.
Mechanism: Varies by class; reduces vascular resistance.
Side effects: Cough (ACEi), swelling (CCB), kidney effects (ARBs).Bone protection (Zoledronic acid or Denosumab)
Class: Antiresorptives.
Dose/time: Zoledronic acid IV every 12 months (osteoporosis) or more often for bone mets; Denosumab SC monthly/6-monthly per indication.
Purpose: Prevent fractures from steroid exposure or bone metastasis.
Mechanism: Inhibits bone breakdown.
Side effects: Low calcium, rare jaw osteonecrosis; dental check advised.Physiologic steroid replacement (when on mitotane or after adrenalectomy)
Class: Hydrocortisone ± fludrocortisone.
Dose/time: Hydrocortisone split doses; fludrocortisone daily if needed.
Purpose: Replace needed hormones when natural production is suppressed.
Mechanism: Provides essential cortisol/aldosterone effects.
Side effects: Dose-related weight gain, blood pressure changes; stress-dose education is vital.
Dietary molecular supplements
Note: No supplement cures ACC. Use only if safe with your medicines.
Vitamin D3 (1000–2000 IU/day, adjust to blood levels)
Function/mechanism: Supports bone formation and immune function; low levels are common with steroid therapy.Calcium (1000–1200 mg/day from food; supplements if advised)
Function: Bone strength, works with vitamin D; avoid excess if high calcium in labs.Omega-3 fatty acids (EPA/DHA ~1 g/day)
Mechanism: Anti-inflammatory lipid mediators; may help appetite and triglycerides.Protein supplementation (whey/plant 20–30 g/day if intake is low)
Mechanism: Provides amino acids for muscle repair during treatment.Probiotics (evidence-based strains; daily as labeled)
Mechanism: Supports gut microbiome during antibiotics/chemo; may reduce diarrhea.Soluble fiber (oats, psyllium 5–10 g/day)
Mechanism: Smooths glucose spikes and supports bowel regularity.Magnesium (200–400 mg/day if low)
Mechanism: Helps cramps and arrhythmia risk if electrolytes are disturbed.Ginger extract (per label; typically 500–1000 mg/day)
Mechanism: May reduce nausea; interacts with blood thinners—check first.Turmeric/Curcumin (standardized extract per label)
Mechanism: Anti-inflammatory signaling; variable absorption; check drug interactions.Green-tea polyphenols (EGCG, per label, avoid if on warfarin)
Mechanism: Antioxidant effects; do not exceed safe doses; watch liver enzymes.
Drugs for “immunity support / regenerative care
There are no approved “stem-cell drugs” to treat ACC itself. The items below are supportive medicines used in cancer care to protect the blood and immune system during intensive therapy. Use only under oncology supervision.
Filgrastim (G-CSF)
Dose: Daily subcutaneous injections after chemo per protocol.
Function/mechanism: Stimulates bone marrow to produce neutrophils; lowers infection risk.
Notes: Can cause bone pain; rare spleen issues.Pegfilgrastim (long-acting G-CSF)
Dose: Single SC dose once per chemo cycle.
Function: Same as filgrastim with convenient dosing.
Notes: Similar side effects.Epoetin alfa (or Darbepoetin alfa)
Dose: SC weekly/biweekly.
Function: Raises red blood cells in selected chemo-related anemia.
Mechanism: Erythropoiesis stimulation.
Notes: Use according to strict guidelines; watch clot risk.Thrombopoietin receptor agonists (Eltrombopag / Romiplostim)
Dose: Oral (eltrombopag) or SC (romiplostim) per platelet counts.
Function: Helps platelets recover in selected cases.
Notes: Monitor liver tests (eltrombopag) and clot risk.IV Immunoglobulin (IVIG) — selected immune deficits
Dose: IV per kg at intervals.
Function: Provides passive antibodies when levels are very low.
Notes: Not routine; used only with clear indications.Seasonal inactivated influenza vaccine & indicated vaccines
Dose: Per national schedule (avoid live vaccines during immunosuppression).
Function: Prevents severe infections during treatment.
Notes: Coordinate timing with oncology.
Surgeries
Open adrenalectomy with en-bloc resection
Procedure: Removal of the adrenal gland and any tissue the tumor has invaded, through a careful open incision.
Why: Gold-standard for localized ACC to achieve clear margins and avoid tumor rupture.Regional lymphadenectomy
Procedure: Removal of nearby lymph nodes.
Why: Staging, and may improve local control.Vena cava thrombectomy (if tumor thrombus present)
Procedure: Specialized surgery to remove tumor extending into the large vein.
Why: Restores blood flow, reduces embolus risk, and controls disease.Metastasectomy (lung/liver lesions in select patients)
Procedure: Surgical removal of limited metastases.
Why: May prolong survival and relieve symptoms in carefully chosen cases.Palliative debulking or ablation
Procedure: Remove part of the tumor, or use ablation/embolization to shrink it.
Why: Reduce pain, bleeding, or hormone excess when cure is not possible.
Preventions and risk-reduction ideas
Know your family risk — seek genetic counseling if ACC or Li-Fraumeni is in your family.
Follow surveillance plans if you carry a high-risk mutation (e.g., scheduled imaging/biomarkers).
Do not misuse anabolic steroids or unregulated “hormone boosters.”
Do not smoke; avoid secondhand smoke.
Maintain healthy weight and regular physical activity.
Control blood pressure and blood sugar; keep regular checkups.
Limit unnecessary radiation exposure; keep records of past imaging.
Use workplace protective measures if exposed to chemicals.
Balanced diet and adequate sleep to support immune health.
Seek care early for hormone-related warning signs.
When to see doctors urgently or soon
Urgently (today): Severe abdominal pain with fever, fainting, chest pain, severe weakness, or signs of adrenal crisis (extreme fatigue, vomiting, very low blood pressure).
Soon (within days): New or worsening Cushing-like features, fast-rising blood pressure or blood sugars, sudden swelling of legs, rapid hair/voice changes, unexplained easy bruising, or a new abdominal mass.
Routinely: Any persistent, unexplained abdominal symptoms or hormone-related changes; if you have a high-risk family history.
What to eat and what to avoid
Lean proteins (fish, eggs, legumes) to protect muscle.
High-fiber carbs (whole grains, beans, vegetables) to steady glucose.
Potassium-rich foods (bananas, oranges, spinach) if your potassium is low — but follow lab results if it is high.
Calcium and vitamin-D foods (dairy or fortified alternatives) for bone health.
Hydration — water or oral rehydration solutions if prone to vomiting/diarrhea.
Limit ultra-processed sweets that spike blood sugar (especially with Cushing’s).
Limit salty foods if you have high blood pressure or aldosterone excess.
Avoid grapefruit and St. John’s wort with many cancer drugs (interactions).
Avoid alcohol excess; it worsens sleep, glucose, and liver strain.
Avoid unverified herbal megadoses; many interact with chemo or mitotane.
Frequently asked questions
Is ACC curable?
Yes, if found early and completely removed. Advanced cases are harder and need multiple treatments.What is the main treatment?
Surgery is the main curative treatment. Mitotane and sometimes chemotherapy or immunotherapy are used when needed.Do all tumors make hormones?
No. About half are functional. Testing shows if hormones are high.Why is biopsy often avoided first?
There is a risk of tumor spread or rupture. If the tumor looks resectable, surgeons often remove it directly.Will I need hormone pills after surgery?
Many people do, especially with mitotane therapy, because it suppresses normal adrenal function.What follow-up will I need?
Regular CT/MRI scans, hormone tests, and clinic visits for several years, most often every 3–6 months at first.Can immunotherapy help ACC?
It helps some people, especially after standard therapy. Response rates vary and side effects must be watched.Is laparoscopic surgery OK?
For suspected ACC, open surgery is generally preferred to avoid tumor rupture; your surgeon will explain why.How big is “concerning” on scans?
Size alone is not diagnosis, but adrenal masses ≥4–6 cm with certain features raise suspicion and need expert care.What if my cortisol is very high before surgery?
Doctors use medicines (ketoconazole, metyrapone, osilodrostat) to control cortisol and reduce surgical risk.Can lifestyle changes shrink the tumor?
No. Lifestyle supports overall health and treatment tolerance but does not cure ACC.Is ACC the same as pheochromocytoma?
No. Pheochromocytoma comes from the adrenal medulla and makes catecholamines; ACC is from the cortex.Should my family be tested?
If you are young, have bilateral disease, or have a family history, genetic counseling can advise on testing.Can ACC return after surgery?
Yes. That is why regular imaging and hormone checks are essential.Where should I get treated?
At a center with experience in adrenal cancers, where surgeons, endocrinologists, medical oncologists, and genetic counselors work together.
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.
