Adrenocortical carcinoma (ACC) is a rare, fast-growing cancer that starts in the outer layer of the adrenal gland, called the cortex. The adrenal cortex makes important hormones, such as cortisol, aldosterone, and androgens. These hormones control stress response, blood pressure, salt balance, and sex traits. ACC can be functioning (making too much hormone) or non-functioning (not making excess hormone). Too much hormone can cause serious body changes, like Cushing’s syndrome (too much cortisol), virilization (extra male traits), or high blood pressure and low potassium. Tumors can grow into nearby tissues and can spread to the liver, lung, bone, or lymph nodes. Doctors stage ACC from I to IV. Early stages are limited to the adrenal. Later stages show large size, invasion, or distant spread. ACC is also called adrenal cortical carcinoma, adrenocortical malignant tumor, or simply adrenal cortex cancer. It can occur at any age but is most common in adults in their 40s–50s and in children under 5 years. Some people have genetic risks, such as Li-Fraumeni syndrome (TP53), Beckwith–Wiedemann syndrome, MEN1, or Lynch syndrome. The main treatment is surgery to remove the tumor. Mitotane and combination chemotherapy are often used. Radiation and targeted or immune treatments may be added in selected cases or trials. Care is best in centers with experience because management is complex and needs a team.
Adrenocortical carcinoma (ACC) is a rare, aggressive cancer that starts in the adrenal cortex, which is the outer layer of the adrenal gland. You have two adrenal glands. They sit on top of your kidneys and make important hormones, such as cortisol, aldosterone, and sex hormones (androgens and estrogens). ACC can make extra hormones (“functional”) or make no extra hormones (“non-functional”). Extra hormones cause body changes like weight gain in the face and trunk, high blood pressure, extra body hair in females, breast enlargement in males, or muscle weakness. Some tumors cause pain or a feeling of fullness in the belly as they grow. Diagnosis uses hormone tests and imaging (CT, MRI, PET). Pathology with Weiss score and Ki-67 helps confirm cancer and predict behavior. Cancer.gov+1PMC
Other names
ACC is also called adrenal cortical carcinoma, adrenocortical cancer, cancer of the adrenal cortex, or simply adrenal cancer (note: “adrenal cancer” can also include other adrenal tumors, so “adrenocortical carcinoma” is more precise). Doctors may say malignant adrenocortical tumor or primary adrenal cortical carcinoma. In children, you may see pediatric adrenocortical carcinoma. When a tumor makes hormones, it may be labeled by what it makes: cortisol-producing, androgen-producing (virilizing), estrogen-producing (feminizing), aldosterone-producing, or mixed hormone–producing. American Cancer SocietyCancer.gov
Types
1) By hormone function
Functional ACC: makes excess hormones (often cortisol, androgens, estrogens, or aldosterone). These cause symptoms such as Cushing features, virilization (in females), feminization (in males), or high blood pressure with low potassium. American Cancer SocietyCancer.gov
Non-functional ACC: does not make extra hormones; symptoms come from mass effect (pain, fullness) or are found incidentally on scans. Medscape
2) By histologic variant (pathology)
Conventional ACC, oncocytic ACC, myxoid ACC, and sarcomatoid ACC are described by the pathologist. Aggressiveness is graded with Weiss criteria and Ki-67 index; higher Ki-67 means a worse outlook. PMCOxford Academic
3) By stage (ENSAT I–IV)
Stage is based on tumor size, local invasion, lymph nodes, and metastasis. Doctors commonly use the ENSAT system. European Society of Endocrinology
Causes
Most ACCs are sporadic (no clear cause). A smaller number happen in people with inherited syndromes or specific gene changes. Below are known and suspected causes/risk factors and disease mechanisms. Each item is a short, self-contained paragraph.
Sporadic tumor with random DNA damage
Many ACCs arise without a family history. Over time, cells in the adrenal cortex collect DNA errors. If key genes controlling growth are damaged, a cancer can start. NCBITP53 (p53) gene mutations
The p53 protein guards against cancer. When TP53 is mutated in adrenal cells, damaged cells can survive and multiply, which can lead to ACC. Somatic TP53 mutations occur in a significant subset of ACCs. BioMed CentralLi-Fraumeni syndrome (germline TP53)
People born with a faulty TP53 (Li-Fraumeni) have a much higher lifetime cancer risk, and ACC is a known tumor in this syndrome, especially in children. NCBIBeckwith–Wiedemann syndrome (11p15/IGF2 dysregulation)
This imprinting disorder alters growth signals. IGF2 overexpression can drive adrenal growth and is linked to pediatric adrenocortical tumors. Ewha Medical JournalLynch syndrome (DNA mismatch repair genes)
Inherited faults in MLH1, MSH2, MSH6, PMS2, EPCAM raise risks for several cancers. ACC is now recognized as a Lynch-associated tumor in some families. PMCOxford AcademicMEN1 gene alterations
MEN1 is a tumor suppressor gene. MEN1 changes are uncommon in ACC but do occur and reflect broader endocrine tumor risk. BioMed CentralCarney complex (PRKAR1A)
Faults in PRKAR1A alter cAMP signaling. Carney complex usually causes benign adrenal nodules (PPNAD) and Cushing syndrome, but rare ACC cases are reported. Oxford AcademicScienceDirectWnt/β-catenin pathway activation (CTNNB1)
Mutations in CTNNB1 and related genes activate growth signals. Wnt/β-catenin activation is common in adrenal tumors and present in a notable fraction of ACCs. PMCZNRF3 loss (Wnt regulation)
ZNRF3 restrains Wnt signaling. When it is lost or mutated, β-catenin activity rises, which can support adrenocortical tumor growth. Oxford AcademicScienceDirectGenomic instability (copy-number changes)
ACC often shows many DNA gains and losses, which disturb growth control and hormone production. This genomic instability helps tumors evolve and spread. BioMed CentralKi-67–high proliferative biology
A high Ki-67 index means cells divide rapidly. While Ki-67 itself is not a cause, tumors with high Ki-67 reflect aggressive, fast-growing biology. Oxford AcademicPediatric genetic susceptibility
Children with germline TP53 or IGF2 pathway abnormalities have increased ACC risk compared with the general population. Cancer.govHormonal microenvironment and chronic stimulation
Abnormal adrenal signaling environments (for example, cAMP pathway dysregulation in Carney complex) can set the stage for tumor formation. Oxford AcademicEpigenetic dysregulation
ACC shows widespread changes in DNA methylation and imprinting (for example at 11p15/IGF2), which can switch growth programs on. PMCRare familial clusters without a known gene
Some families have more than one case of ACC but no single identified gene yet; this suggests additional undiscovered inherited factors. Lippincott JournalsDefects in DNA repair beyond Lynch (general concept)
Broader problems in DNA repair pathways can allow mutations to build up in adrenal cells, making cancer more likely over many years. Lippincott JournalsCooperating hits in animal models (p53 + Wnt)
Research in models shows that a combination of p53 loss plus Wnt/β-catenin activation drives malignant adrenal growth more than either event alone. NatureMEN1 pathway and chromatin control (low frequency)
Low-frequency MEN1 changes point to altered chromatin regulation as one of several paths to ACC development. BioMed CentralPRKAR1A/cAMP signaling (rare malignant transformation)
In Carney complex, the same cAMP pathway that drives benign nodules can rarely progress toward carcinoma when other hits accumulate. PubMedGeneral cancer risks (age, chance, unknown exposures)
Many adult cases have no clear trigger. Age-related DNA damage and unknown environmental factors likely play a role in sporadic disease. NCBI
Symptoms
Abdominal or flank pain/fullness — a growing mass can stretch tissues or press on nearby organs. Cancer.gov
Unplanned weight loss or appetite loss — common with advanced cancers or hormone disturbances. Mayo Clinic
High blood pressure — extra cortisol or aldosterone can raise blood pressure. Cancer.gov
Low potassium (muscle cramps/weakness) — from aldosterone excess; may cause fatigue or palpitations. Mayo Clinic
Round, puffy face; central weight gain; thin skin/easy bruising — classic Cushing features from cortisol excess. Mayo Clinic
New or worsening diabetes/high blood sugar — cortisol raises glucose. Mayo Clinic
Proximal muscle weakness — especially in shoulders/hips due to cortisol excess. NCBI
Excess facial/body hair (females) — androgen excess (virilization) with acne and voice deepening. American Cancer Society
Menstrual changes or amenorrhea (females) — androgens or cortisol can disrupt cycles. American Cancer Society
Breast enlargement and tenderness (males) — estrogen-secreting tumors cause gynecomastia and decreased libido. American Cancer Society
Erectile dysfunction (males) — from estrogen excess or cortisol effects. American Cancer Society
Early puberty changes (children) — androgens/estrogens can trigger early hair growth or rapid growth. Cancer.gov
Back pain — tumor mass or local invasion can irritate nerves or muscles. Mayo Clinic
Incidental finding on scans — some ACCs are found during imaging for other reasons. Medscape
General fatigue and anxiety — common with hormone excess and cancer-related stress. adrenal.com
Diagnostic tests
A) Physical examination
Targeted endocrine physical exam
Doctor checks for Cushing features (round face, easy bruising), skin thinning, acne, and muscle wasting. This points toward hormone-producing tumors. Mayo ClinicBlood pressure and general vitals
High blood pressure and rapid heart rate suggest cortisol or aldosterone excess. Persistent hypertension triggers hormone testing. Cancer.govAbdominal palpation
A careful belly exam can reveal tenderness or a palpable mass if the tumor is large. This guides imaging. Cancer.govPediatric pubertal staging
In children, doctors assess for early puberty signs from excess sex hormones, which can point to ACC. PMC
B) Manual/bedside assessments
Orthostatic blood pressure measurement
BP checked lying and standing helps spot volume expansion or cortisol effects on circulation. It complements lab work. Mayo ClinicManual muscle strength testing
Simple bedside testing of shoulder/hip strength detects proximal weakness of Cushing syndrome. NCBIFerriman–Gallwey scoring for hirsutism
A visual scoring of hair growth in females quantifies androgen effects and supports biochemical testing. (Standard endocrine practice.) American Cancer SocietyAnthropometrics (BMI/waist circumference)
Central adiposity, thin limbs, and weight changes support cortisol excess and help monitor response to therapy. Mayo Clinic
C) Laboratory & pathological tests
1-mg overnight dexamethasone suppression test (DST)
You take dexamethasone at night; morning cortisol should be low. Failure to suppress suggests cortisol excess that needs full workup. PMC24-hour urinary free cortisol or late-night salivary cortisol
Either test confirms abnormal cortisol production when DST is positive or symptoms are strong. Cancer.govAndrogen/estrogen panel (DHEA-S, testosterone/androstenedione, estradiol)
High adrenal androgens (DHEA-S) or unexpected estrogens support a functional ACC and help track treatment. Boston Medical CenterAldosterone–renin ratio (with confirmatory testing)
Screening for primary aldosteronism when hypertension and low potassium are present. Confirms aldosterone-producing tumors. Boston Medical Center
Pathology note: When tissue is available (usually after surgery), Weiss score and Ki-67 index help confirm malignancy and predict risk; higher Ki-67 means worse prognosis. Biopsy is generally avoided unless it will change management in unresectable disease. PubMedOxford Academic
D) Electrodiagnostic/functional tests
Electrocardiogram (ECG)
Looks for effects of low potassium (from aldosteronism) or cortisol-related heart strain (arrhythmias, QT changes). Mayo ClinicAmbulatory blood pressure monitoring (ABPM) or Holter
24-hour recordings document persistent hypertension or rhythm issues related to hormone excess. Mayo ClinicElectromyography (EMG) when severe weakness is present
Detects steroid-related myopathy patterns in people with Cushing syndrome from ACC. NCBIGlucose tolerance monitoring (glucometer profiles)
Tracks cortisol-related hyperglycemia patterns to support diagnosis and guide diabetes care. Mayo Clinic
E) Imaging tests
Adrenal-protocol CT scan
CT with density and washout features helps tell benign from malignant adrenal masses and maps local spread. ScienceDirectMRI (chemical-shift and contrast)
MRI characterizes adrenal tissue (lipid-rich vs lipid-poor) and shows invasion into vessels or liver. It is as effective as CT for many questions. ScienceDirectFDG PET-CT for staging
PET-CT detects metabolically active disease and distant metastases, helping stage and plan therapy. ESMOChest CT and targeted liver imaging
Because ACC can spread to lungs and liver, dedicated scans look for metastases before treatment.
Non-pharmacological treatments
Pre- and post-operative breathing exercises (physiotherapy).
Description: Simple, taught moves like diaphragmatic breathing and incentive spirometry done several times a day before and after surgery.
Purpose: Reduce lung problems after anesthesia and pain.
Mechanism: Deep breaths reopen small air sacs, improve oxygen flow, and clear mucus.
Benefits: Fewer complications like pneumonia, better oxygen levels, faster walking, and shorter hospital stay.Early mobilization and graded walking (physiotherapy).
Description: Start sitting, standing, and walking soon after surgery or when safe during chemo. Increase steps daily.
Purpose: Prevent clots, muscle loss, and deconditioning.
Mechanism: Muscle pump improves blood return; movement preserves strength and balance.
Benefits: Less fatigue, lower risk of DVT, improved bowel function, and quicker return to daily life.Core and posture training (physiotherapy).
Description: Gentle core activation, pelvic tilts, wall slides, and postural cues.
Purpose: Support trunk after flank or midline incisions and protect the back.
Mechanism: Stronger core stabilizes spine and incision area, reducing strain.
Benefits: Less pain, safer movement, and better function for lifting, coughing, and deep breathing.Lymphatic drainage techniques and edema control (physiotherapy).
Description: Elevation, light massage, and compression when advised.
Purpose: Reduce swelling in legs or abdomen after major surgery or with steroids.
Mechanism: Improves lymph flow and shifts fluid back into circulation.
Benefits: Less tightness, easier movement, and better wound healing.Pelvic floor and abdominal wall protection training (physiotherapy).
Description: Log-rolling, cough support with a pillow, and step-by-step safe lifting.
Purpose: Protect the incision and prevent hernia.
Mechanism: Reduces sudden pressure on healing tissues.
Benefits: Safer daily movement and more comfort.Energy-conservation pacing (physiotherapy/rehab).
Description: Plan the day with rest between tasks, sit for tasks when possible, and use a step-by-step list.
Purpose: Manage cancer-related fatigue.
Mechanism: Balances load with recovery to avoid “boom-and-bust” cycles.
Benefits: More stable energy and better ability to keep up with care.Progressive resistance exercise (physiotherapy).
Description: Light bands or body-weight moves two to three days per week, as tolerated.
Purpose: Maintain muscle during treatment.
Mechanism: Stimulates muscle protein synthesis and neuromuscular function.
Benefits: Improved strength, independence, and glucose control.Mindful breathing and body scan (mind-body).
Description: 10–15 minutes daily of slow breathing and scanning body sensations.
Purpose: Reduce anxiety, pain, and sleep problems.
Mechanism: Activates the parasympathetic system and reduces cortisol spikes.
Benefits: Calmer mood, better pain control, and improved sleep.Guided imagery for symptom control (mind-body).
Description: Audio guidance to visualize safe, strong, healing scenes.
Purpose: Lower nausea, fear, and treatment stress.
Mechanism: Shifts attention and reduces autonomic arousal.
Benefits: Less nausea and distress; more control.Cognitive behavioral coping skills (mind-body/educational).
Description: Short lessons and practice to reframe unhelpful thoughts about illness.
Purpose: Improve mood and adherence.
Mechanism: Thought–feeling–behavior links are adjusted toward helpful patterns.
Benefits: Lower depression and anxiety; better self-care.Acceptance and commitment therapy micro-skills (mind-body).
Description: Brief exercises to accept difficult sensations while acting on values.
Purpose: Reduce struggle with uncertainty.
Mechanism: Psychological flexibility improves stress handling.
Benefits: More steady coping and quality of life.Sleep hygiene curriculum (educational).
Description: Set sleep/wake time, low light at night, limit screens, and wind-down routine.
Purpose: Improve sleep quality.
Mechanism: Resets circadian rhythm and lowers arousal.
Benefits: Better energy, mood, and immune function.Treatment plan education and red-flag training (educational).
Description: Clear teaching on meds, wound care, steroid rules, and when to call.
Purpose: Prevent avoidable crises.
Mechanism: Knowledge increases timely action.
Benefits: Fewer ER visits and safer recovery.Steroid safety education (educational).
Description: Teach about adrenal suppression, stress-dose steroids, and medical alert ID.
Purpose: Avoid adrenal crisis.
Mechanism: Ensures correct extra dosing during fever, surgery, or trauma.
Benefits: Life-saving prevention of hypotension and shock.Nutrition basics and label literacy (educational).
Description: Teach balanced meals, sodium/potassium targets when relevant, and reading labels.
Purpose: Support healing and hormone issues.
Mechanism: Food choices help blood pressure, glucose, and weight.
Benefits: Better strength, fewer side effects, and improved outcomes.Smoking cessation program (other supportive).
Description: Counseling plus nicotine replacement if needed.
Purpose: Improve wound healing and lung health.
Mechanism: Removes carbon monoxide and toxins that harm blood vessels.
Benefits: Fewer complications and better overall survival.Alcohol moderation or abstinence plan (other supportive).
Description: Clear limits or a stop plan with support.
Purpose: Protect liver during chemo and hormone therapy.
Mechanism: Reduces inflammation and drug interactions.
Benefits: Safer treatment and better sleep.Vaccination review (other supportive).
Description: Update inactivated vaccines (flu, COVID-19, pneumococcal) before chemo if possible.
Purpose: Lower infection risk.
Mechanism: Prepares immune memory before suppression.
Benefits: Fewer severe infections.Infection-prevention skills (other supportive).
Description: Hand hygiene, food safety, mask in crowded settings, and oral care.
Purpose: Prevent neutropenic infections.
Mechanism: Cuts exposure to pathogens.
Benefits: Lower fever episodes and hospital stays.Pain self-management toolkit (other supportive).
Description: Heat/cold packs, relaxation, activity pacing, and positioning.
Purpose: Reduce pain and cut opioid use when possible.
Mechanism: Alters pain signaling and reduces muscle spasm.
Benefits: Better function and mood.Financial and work counseling (other supportive).
Description: Help with leave, insurance, and disability paperwork.
Purpose: Reduce money and job stress.
Mechanism: Early planning avoids crises.
Benefits: Better adherence and less anxiety.Fertility and family planning counseling (other supportive).
Description: Discuss options before treatment if relevant.
Purpose: Protect future choices.
Mechanism: Timely referral to fertility specialists.
Benefits: Preserves options and reduces regret.Genetic counseling (other supportive).
Description: Review personal and family risk for TP53, MEN1, etc.
Purpose: Guide testing and family screening.
Mechanism: Risk assessment and informed testing.
Benefits: Early detection for relatives and tailored follow-up.Psycho-oncology referral (other supportive).
Description: Regular sessions with a clinician trained in cancer care.
Purpose: Manage depression, fear, or PTSD-like symptoms.
Mechanism: Evidence-based therapy and medication when needed.
Benefits: Better quality of life and treatment completion.Palliative care integration (other supportive).
Description: Symptom-focused care at any stage, not only end of life.
Purpose: Control pain, nausea, fatigue, and stress.
Mechanism: Multidisciplinary support, advance-care planning.
Benefits: Less suffering and often longer life.
Drug treatments
(Each includes a short description, class, typical dosing/time notes, purpose, mechanism, and key side effects. Doses are typical adult references—final decisions must be individualized by the oncology team.)
Mitotane.
Class: Adrenolytic/steroidogenesis inhibitor.
Dose/time: Often started around 1–2 g/day and slowly titrated toward 6–10 g/day; target serum level commonly 14–20 mg/L; taken for months to years if tolerated.
Purpose: Main systemic drug for ACC; also used after surgery to reduce recurrence risk in selected high-risk cases.
Mechanism: Selectively damages adrenal cortical cells and blocks cortisol synthesis.
Side effects: Nausea, diarrhea, fatigue, neurocognitive slowing, liver enzyme rise, very strong drug interactions (induces CYP3A4), and adrenal insufficiency requiring steroid replacement.EDP-M regimen (Etoposide, Doxorubicin, Cisplatin plus Mitotane).
Class: Combination cytotoxic chemotherapy plus adrenolytic therapy.
Dose/time: Cycles about every 3–4 weeks; mitotane continued.
Purpose: First-line regimen for advanced/metastatic ACC in many guidelines.
Mechanism: DNA damage (cisplatin), topoisomerase inhibition (etoposide, doxorubicin), plus mitotane adrenolysis.
Side effects: Low blood counts, nausea, hair loss, kidney and nerve toxicity (cisplatin), heart toxicity risk (doxorubicin), infections; needs growth-factor and anti-emetic support.Streptozocin-based regimens.
Class: Alkylating nitrosourea.
Dose/time: Given IV in cycles; sometimes with mitotane.
Purpose: Alternative regimen when EDP-M is not suitable.
Mechanism: DNA alkylation causing tumor cell death.
Side effects: Kidney toxicity, nausea, low counts; careful monitoring needed.Gemcitabine ± Capecitabine.
Class: Antimetabolites.
Dose/time: IV gemcitabine days 1 and 8; capecitabine oral twice daily 14/21 days, cycles repeat.
Purpose: Option after progression on first-line therapy.
Mechanism: Inhibits DNA synthesis.
Side effects: Fatigue, low counts, hand-foot syndrome (capecitabine), liver enzymes.Temozolomide.
Class: Oral alkylating agent.
Dose/time: Commonly days 1–5 every 28 days.
Purpose: Off-label salvage option in some centers.
Mechanism: Methylates DNA, leading to tumor cell death.
Side effects: Low counts, nausea, fatigue; rare serious infections.Paclitaxel (selected cases).
Class: Microtubule inhibitor.
Dose/time: Weekly or q3-week schedules.
Purpose: Later-line palliative option in refractory disease.
Mechanism: Stabilizes microtubules, halting cell division.
Side effects: Neuropathy, low counts, hair loss, hypersensitivity.Cabozantinib or Sunitinib (selected refractory cases).
Class: Multikinase inhibitors (VEGFR/MET/others).
Dose/time: Oral daily; dose often adjusted for tolerance.
Purpose: Off-label in trial-ineligible, heavily pretreated disease.
Mechanism: Anti-angiogenic and anti-proliferative signaling blockade.
Side effects: Fatigue, hand-foot syndrome, diarrhea, hypertension, thyroid dysfunction.Pembrolizumab (immune checkpoint inhibitor).
Class: Anti-PD-1 antibody.
Dose/time: IV every 3–6 weeks.
Purpose: Option in selected refractory ACC, sometimes with MSI-H/TMB-high or other biomarkers.
Mechanism: Restores T-cell activity against tumor.
Side effects: Immune-related events (thyroiditis, hepatitis, colitis, pneumonitis); needs prompt steroids if severe.Nivolumab ± Ipilimumab.
Class: Anti-PD-1 ± anti-CTLA-4.
Dose/time: IV per standard schedules.
Purpose: Considered in clinical-trial or salvage settings.
Mechanism: Dual checkpoint blockade to boost anti-tumor immunity.
Side effects: Higher risk of immune-related toxicities (skin, gut, endocrine).Metyrapone.
Class: Steroidogenesis inhibitor.
Dose/time: Oral divided doses; rapid titration for cortisol control.
Purpose: Control of Cushing’s syndrome due to functioning ACC.
Mechanism: Blocks 11-beta-hydroxylase to reduce cortisol.
Side effects: Hypertension, low potassium, acne, hirsutism, edema; needs monitoring.Osilodrostat or Ketoconazole (steroid control).
Class: Steroidogenesis inhibitors.
Dose/time: Oral, titrated to cortisol targets.
Purpose: Lower cortisol excess when mitotane is not enough or not yet therapeutic.
Mechanism: Blocks cortisol-making enzymes (CYP11B1; multiple CYPs for ketoconazole).
Side effects: Liver enzyme rise (especially ketoconazole), adrenal insufficiency, QT prolongation (osilodrostat).Etomidate (ICU use).
Class: IV anesthetic with steroidogenesis inhibition.
Dose/time: Continuous low-dose infusion.
Purpose: Emergency control of severe hypercortisolism.
Mechanism: Rapid block of 11-beta-hydroxylase.
Side effects: Sedation, adrenal insufficiency; ICU monitoring required.Spironolactone or Eplerenone.
Class: Mineralocorticoid receptor antagonists.
Dose/time: Oral daily; titrate to blood pressure and potassium.
Purpose: Treat aldosterone excess from functioning ACC.
Mechanism: Blocks aldosterone action in kidney.
Side effects: High potassium, low blood pressure; spironolactone can cause breast tenderness or menstrual changes.Abiraterone (selected cases).
Class: CYP17 inhibitor.
Dose/time: Oral daily with steroid support.
Purpose: Control excess androgen or cortisol drive in selected ACC cases.
Mechanism: Blocks androgen and cortisol synthesis upstream.
Side effects: Low potassium, fluid retention, liver enzyme rise; needs steroid replacement.Supportive antiemetics and growth-factor support with chemo.
Class: 5-HT3 antagonists, NK1 antagonists, dexamethasone (with caution if cortisol control is needed), and G-CSF agents.
Dose/time: Per cycle-specific protocols.
Purpose: Prevent nausea and maintain white counts.
Mechanism: Receptor blockade of emesis pathways; bone-marrow stimulation.
Side effects: Headache/constipation (antiemetics), bone pain (G-CSF).
Dietary molecular supplements
(Supportive only; always discuss interactions—mitotane and many drugs interact via CYP enzymes.)
High-protein whey or plant protein (20–30 g/day).
Function: Preserve muscle and support healing.
Mechanism: Provides essential amino acids for repair.
Note: Adjust if kidney issues; space away from chemo if it worsens nausea.Omega-3 fatty acids (EPA/DHA 1–2 g/day).
Function: May help inflammation and weight loss with cachexia.
Mechanism: Membrane and eicosanoid effects.
Note: Bleeding risk is low but discuss if platelets are low.Vitamin D3 (1,000–2,000 IU/day; higher only if deficient per labs).
Function: Bone and immune support, especially with steroid use.
Mechanism: Calcium balance and immune modulation.
Note: Check levels; avoid excess.Calcium (total 1,000–1,200 mg/day from diet; supplement to reach target).
Function: Protect bone when on steroids.
Mechanism: Supports bone mineralization.
Note: Split doses; monitor if kidney stones.Magnesium (200–400 mg/day).
Function: Helps muscle, heart rhythm, and sleep.
Mechanism: Enzyme cofactor.
Note: Diarrhea if too high; monitor if on cisplatin (can cause low magnesium).Probiotics (evidence varies; choose well-studied strains).
Function: Gut support during antibiotics/chemo.
Mechanism: Microbiome balance.
Note: Avoid if severe neutropenia or central lines unless your team approves.Soluble fiber (oats/psyllium 5–10 g/day).
Function: Bowel regularity; cholesterol support.
Mechanism: Gel-forming stool bulk.
Note: Separate from oral meds to avoid absorption issues.B-complex (at RDA levels).
Function: Nerve and energy metabolism support.
Mechanism: Cofactor roles.
Note: Avoid mega-doses; can change urine color.Ginger extract or tea (up to 1–2 g/day).
Function: Nausea relief.
Mechanism: 5-HT3 modulation and gastric motility.
Note: Possible interactions with anticoagulants; check with team.Curcumin (turmeric standardized, e.g., 500–1,000 mg/day).
Function: Anti-inflammatory support.
Mechanism: NF-κB and cytokine pathways.
Note: Can interact with chemo and liver enzymes; only use if team approves.
Immunity-booster / regenerative / stem-cell–related” drugs
There are no approved “stem cell drugs” for ACC. Unsafe or unregulated stem-cell products should be avoided. Here are medically accepted supportive biologics used to protect the blood system during treatment:
Filgrastim (G-CSF).
Dose: Commonly 5 mcg/kg/day SC for several days post-chemo.
Function/mechanism: Stimulates neutrophil production to reduce infection risk.Pegfilgrastim.
Dose: 6 mg SC once per chemo cycle (timing per protocol).
Function/mechanism: Long-acting G-CSF to prevent severe neutropenia.Darbepoetin alfa or Epoetin alfa (ESAs).
Dose: Given SC/IV on set schedules if chemo-induced anemia and criteria met.
Function/mechanism: Stimulates red blood cell production to reduce transfusions.Romiplostim or Eltrombopag (for selected thrombocytopenia).
Dose: Titrated weekly (romiplostim) or daily (eltrombopag) per counts.
Function/mechanism: Thrombopoietin receptor agonists raise platelet counts.IVIG (intravenous immunoglobulin) in special cases.
Dose: Weight-based courses.
Function/mechanism: Provides pooled antibodies when serious immune defects or infections occur.Vaccines (inactivated) scheduled appropriately.
Dose: Per national schedules before chemo if possible.
Function/mechanism: Builds protective immunity against key pathogens.
Surgeries
Open adrenalectomy with en-bloc resection.
Procedure: Remove the adrenal tumor, often through an open incision, sometimes with part of adjacent organs if invaded.
Why: Main curative option for localized ACC; open approach lowers the risk of tumor spill.Regional lymphadenectomy.
Procedure: Remove nearby lymph nodes.
Why: Improve staging accuracy and may improve local control.Inferior vena cava (IVC) thrombectomy (when tumor thrombus present).
Procedure: Vascular surgery to remove tumor extension into the IVC.
Why: Restore blood flow and achieve complete resection.Metastasectomy (liver, lung, or others) in selected cases.
Procedure: Remove limited metastatic sites.
Why: May prolong survival in carefully chosen patients with slow or limited spread.Debulking/palliative surgery.
Procedure: Remove bulk of tumor when cure is not possible.
Why: Reduce hormone excess, pain, or pressure symptoms to improve quality of life.
Prevention strategies
Genetic counseling and testing when family history suggests TP53/MEN1/BWS/Lynch.
Regular surveillance for high-risk gene carriers per specialist guidance.
Avoid anabolic steroid misuse and unnecessary exogenous hormones.
No smoking; smoking harms healing and overall survival.
Maintain healthy weight and activity, which supports hormone balance and immune health.
Limit alcohol to protect the liver during treatment.
Occupational safety (reduce exposure to industrial carcinogens).
Minimize unnecessary radiation from imaging; use justified tests only.
Rapid evaluation of hormone symptoms (unexplained weight gain, bruising, virilization, resistant hypertension, low potassium).
Care in experienced centers; expert surgery reduces recurrence risk.
When to see doctors
Urgent now: Fever ≥38.0°C, chills, severe vomiting, sudden weakness or confusion, severe abdominal pain, shortness of breath, chest pain, bleeding, or signs of adrenal crisis (very low blood pressure, severe dizziness, vomiting with inability to keep steroids down).
Soon (within 24–48 hours): New or fast-worsening swelling, leg pain/redness (possible clot), yellow eyes/skin, painful urination, uncontrolled pain, or new neurological symptoms.
Routine appointment: Any new hormone-related changes (rapid weight change, new stretch marks, facial rounding, acne/hair growth in women, new high blood pressure, muscle weakness), medication side effects, questions about surgery, chemo, or fertility, and to plan vaccinations or nutrition.
What to eat and what to avoid
Eat balanced meals with lean protein, whole grains, fruits, and vegetables to support healing.
Aim for enough protein daily (about 1.0–1.2 g/kg/day; your team may adjust).
If cortisol is high, choose lower-sodium foods to help blood pressure and edema.
If aldosterone is high with low potassium, add potassium-rich foods (bananas, oranges, beans) under clinician guidance.
Drink enough fluids unless told otherwise; this helps kidneys, especially with cisplatin.
Limit alcohol; it stresses the liver and worsens sleep.
Food safety first: wash produce, cook meats well, avoid raw seafood during neutropenia.
Small, frequent meals and bland choices on chemo days to reduce nausea.
Discuss supplements before starting; many interact with mitotane and chemo.
Stable weight goals: if losing weight, ask for a nutrition plan with calorie-dense, high-protein snacks.
FAQs
1) Is ACC the same as “adrenal cancer”?
ACC is the main type of adrenal cortex cancer. The adrenal medulla has different tumors (like pheochromocytoma), which are not ACC.
2) Can ACC make too much hormone?
Yes. Many ACCs are functioning and cause Cushing’s syndrome, virilization, or aldosterone excess.
3) What is the first treatment for localized ACC?
Surgery by an experienced team using an open approach to fully remove the tumor.
4) Do I always need medicine after surgery?
High-risk patients may receive mitotane after surgery to lower recurrence risk. Your team decides based on stage and pathology.
5) What if my ACC has spread?
Systemic therapy (like EDP-M) is common. Some patients also have metastasectomy if disease is limited and slow.
6) Will I need steroids?
Often yes, especially on mitotane or after adrenal removal. You may carry a steroid card and know stress-dose rules.
7) Are immunotherapies used?
They may be used in selected advanced cases or trials. Benefits vary; side effects can affect many organs.
8) Can targeted pills help?
Some multikinase inhibitors are tried in later lines. Evidence is limited; talk with your oncologist.
9) How common is ACC?
It is rare. Because it’s rare, care in a specialty center is very important.
10) What follow-up is needed after surgery?
Regular scans and hormone tests. The schedule is set by stage and risk.
11) Can children get ACC?
Yes, especially with certain syndromes. Pediatric specialists should guide care.
12) How long is mitotane treatment?
Often many months to years if tolerated and if it is helping. Blood levels are monitored.
13) Can I get pregnant after ACC treatment?
Discuss with your team. Some treatments affect fertility. Early fertility counseling is best.
14) What can I do to help myself today?
Do your breathing and walking plan, follow infection prevention, keep good sleep habits, and ask questions early.
15) Where can I learn about trials?
Ask your oncology team about clinical trials. Trials may offer new options and expert care.Y
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.
