Malignant Adrenal Gland Neoplasm

A malignant adrenal gland neoplasm is a cancer that starts in one of the two small glands that sit on top of the kidneys. Each adrenal gland has two parts. The cortex makes steroid hormones (like cortisol, aldosterone, and sex hormones). The medulla makes stress hormones (adrenaline and noradrenaline). When cells in either part grow out of control and spread to other places, it is called malignant (cancer). These tumors may make extra hormones, which cause body changes, or they may stay “silent” and grow quietly until they are large. Treatment and outlook depend on the type, the size, whether it has spread, and whether it makes hormones.

A malignant adrenal gland neoplasm is a cancer that starts in one of the two adrenal glands sitting above the kidneys. The adrenals make hormones that control blood pressure, salt balance, stress response, sex traits, and metabolism. When cells in the adrenal cortex (outer layer) or medulla (inner core) grow out of control, they can form an aggressive tumor that may make too much hormone and may spread to other organs like the liver, lungs, or bones. Symptoms come from the tumor’s growth (pain, mass effect) or from extra hormones (high blood pressure, diabetes, weight changes). Diagnosis needs imaging, blood/urine hormone tests, and careful staging. Treatment often combines surgery, medicines such as mitotane or chemotherapy, and supportive care. Early detection and complete surgical removal offer the best chance of long-term control.

Other names

Doctors may use several names for this condition. “Adrenocortical carcinoma (ACC)” means cancer starting in the adrenal cortex. “Malignant pheochromocytoma” means a cancerous tumor from the adrenal medulla (chromaffin cells) that can spread. In children, neuroblastoma or ganglioneuroblastoma may arise in the adrenal medulla and behave like cancer. You may also hear “primary adrenal cancer” for tumors that start in the adrenal itself, and “adrenal metastasis” for cancers that spread to the adrenal from somewhere else (like lung, kidney, or melanoma). Some doctors group medullary cancers with “paraganglioma” when similar tumors start outside the adrenal but share features.

Types

1. Adrenocortical carcinoma (ACC). A rare cancer from the adrenal cortex. It may make cortisol, aldosterone, or sex hormones, or be non-functioning.

2. Malignant pheochromocytoma. A catecholamine-producing medullary tumor that has spread or invades nearby tissues; it can cause attacks of severe high blood pressure.

3. Neuroblastoma (pediatric). A childhood tumor of immature nerve cells, often starting in the adrenal medulla; can range from low to high risk.

4. Ganglioneuroblastoma. A mixed tumor with both mature and immature nerve elements; behavior varies.

5. Primary adrenal lymphoma (rare). Cancer of lymphocytes that can involve one or both adrenal glands.

6. Adrenal sarcomas (very rare). Cancers from connective tissue in the adrenal region (e.g., angiosarcoma).

7. Secondary (metastatic) adrenal involvement. Not a primary adrenal cancer but common; cancers from lung, kidney, colon, melanoma, and others can spread to adrenals.

8. Collision tumors (rare). Two different tumors co-existing in one adrenal (e.g., metastasis plus adenoma).

In everyday practice, the two main primary malignant adrenal tumors clinicians think about are ACC (cortex) and malignant pheochromocytoma (medulla).

Causes / risk factors

Note: For many adrenal cancers, a single direct “cause” is unknown. The items below are known genetic syndromes, mutations, and risk factors associated with higher risk.

1. Li-Fraumeni syndrome (TP53 mutation). Strongly raises the risk of ACC, especially in children and in certain regions like southern Brazil.

2. Beckwith–Wiedemann spectrum / 11p15 imprinting defects. Overgrowth syndrome linked to pediatric adrenal tumors; IGF2 dysregulation is common.

3. Familial ACC due to IGF2 overexpression. IGF2 drives cell growth in many ACCs.

4. Multiple Endocrine Neoplasia type 2 (RET mutation). Raises risk of pheochromocytoma; some become malignant.

5. Von Hippel–Lindau (VHL) disease. Predisposes to pheochromocytoma/paraganglioma; cancerous behavior possible.

6. Neurofibromatosis type 1 (NF1). Increases risk of pheochromocytoma; occasionally malignant.

7. SDHB / SDHD / SDHC mutations. Succinate dehydrogenase gene defects are linked with paragangliomas/pheochromocytomas and higher malignancy risk (especially SDHB).

8. MAX and TMEM127 mutations. Newer susceptibility genes for pheochromocytoma/paraganglioma; malignancy can occur.

9. Mismatch repair deficiency (Lynch-spectrum genes). A subset of ACCs shows MMR defects, which can promote cancer development.

10. Carney complex (PRKAR1A). Adrenal cortical disease (PPNAD) with altered cAMP signaling; rare links to malignancy.

11. Long-standing hormonal stimulation. Chronic ACTH drive is a proposed contributor to cortical tumorigenesis (evidence suggestive, not definitive).

12. Founder mutations (e.g., TP53 R337H in Brazil). Specific population variants markedly raise pediatric and adult ACC risk.

13. Prior radiation exposure. Ionizing radiation is a general carcinogen; specific adrenal data are limited but considered a risk.

14. Environmental carcinogens. Some chemicals may contribute to DNA injury (data in adrenal cancer are limited).

15. Age extremes. ACC peaks in children under 5 and adults in their 40s–60s; pheochromocytoma more often in adults.

16. Female sex (for ACC). ACC is slightly more common in females in many series.

17. High birth weight / overgrowth states in childhood. Seen with BWS, which is linked to adrenal tumors.

18. Family history of pheochromocytoma/paraganglioma. Suggests inherited gene mutation risk.

19. Hypoxia-pathway dysregulation (VHL/HIF). Drives growth and angiogenesis in medullary tumors.

20. General cancer susceptibility background. Some people carry multiple low-penetrance variants that modestly raise risk.

Symptoms and signs

1. High blood pressure (steady or in attacks). Hormone-producing tumors, especially pheochromocytoma or aldosterone-producing cancers, can cause severe or sudden spikes in blood pressure.

2. Headache, palpitations, and sweating spells. Classic “triad” for catecholamine excess; can come in sudden episodes.

3. Rapid heartbeat or tremor. Extra adrenaline-like hormones can make the heart race and hands shake.

4. Weight gain with round face and central fat. Too much cortisol (Cushing’s) from a cortical cancer can change body shape.

5. Purple stretch marks and easy bruising. Cortisol thins the skin and damages tiny blood vessels.

6. Muscle weakness. Cortisol breaks down muscle; low potassium from aldosterone also causes weakness and cramps.

7. New or worsening diabetes and high cholesterol. Cortisol raises blood sugar and alters fats in the blood.

8. Bone loss and back pain. High cortisol weakens bones; large adrenal masses can also cause pressure discomfort.

9. Severe low potassium (hypokalemia). Aldosterone excess wastes potassium, causing fatigue, cramps, and sometimes heart rhythm problems.

10. Excess hair growth, acne, or deepening voice in women. Androgen excess from the tumor can cause virilization.

11. Breast enlargement or low libido in men. Estrogen-secreting tumors can feminize male patients.

12. Unintentional weight loss and fatigue. Common cancer-related symptoms even when hormones are normal.

13. Abdominal fullness or flank pain. A large tumor can press on nearby organs.

14. Anxiety or panic feelings. Surges of catecholamines can feel like panic attacks.

15. Symptoms from spread (metastasis). Cough or chest pain (lung), bone pain (bone), or neurologic symptoms if other organs are involved.

Diagnostic tests

(Grouped as Physical Exam, Manual Tests, Lab/Pathology, Electro-diagnostic, and Imaging. Each item is explained in simple terms.)

A) Physical exam

1. Vital signs at rest. The clinician checks blood pressure and heart rate. Long-standing high blood pressure or a fast pulse can point to hormone-producing tumors.

2. Skin and fat distribution. Purple stretch marks, thin skin, bruises, acne, or “moon face” suggest cortisol excess.

3. Body hair and sexual features. Extra facial/body hair in women or breast enlargement in men suggests abnormal sex hormone production.

4. General appearance and strength. Muscle wasting, central obesity, and weakness fit with steroid excess.

5. Abdominal exam. The doctor gently presses to detect masses or tenderness that could reflect a large adrenal tumor or liver enlargement.

B) Manual/bedside tests

6. Orthostatic blood pressure and heart rate. Measurements lying and standing can uncover large swings caused by catecholamines or volume shifts from aldosterone.

7. Manual muscle strength testing. Simple bedside tests of leg and arm strength help detect steroid-related myopathy or weakness from low potassium.

8. Ferriman–Gallwey scoring for hirsutism. A visual scoring system grades excess hair growth in women and supports the diagnosis of androgen excess.

C) Laboratory and pathology tests

9. Overnight 1-mg dexamethasone suppression test (DST). A small steroid pill at night should suppress morning cortisol; failure to suppress suggests autonomous cortisol production by a tumor.

10. 24-hour urinary free cortisol. Measures total cortisol over a day; high values support Cushing’s syndrome from a functioning adrenal cancer.

11. Late-night salivary cortisol. Saliva collected late at night should be low; high levels are a simple screen for cortisol excess.

12. Plasma ACTH level. Low ACTH with high cortisol points to an adrenal source (like ACC). High ACTH suggests a pituitary or ectopic source.

13. Aldosterone-renin ratio (ARR) with electrolytes. A high ARR plus low potassium supports aldosterone excess from an adrenal tumor.

14. Plasma free or urinary fractionated metanephrines. Best biochemical tests for pheochromocytoma; very sensitive for catecholamine-producing tumors.

15. Sex-steroid panel (DHEA-S, testosterone, estradiol). High adrenal androgens (like DHEA-S) or unexpected estrogen levels suggest a hormone-secreting cortical cancer.

Pathology note: When the tumor is removed, the pathologist uses special scoring systems (e.g., Weiss/Helsinki for ACC, PASS for pheochromocytoma) and markers like SF-1 and Ki-67 to judge malignancy and growth rate.

D) Electro-diagnostic tests

16. 12-lead electrocardiogram (ECG). Looks for fast rhythms, strain from high blood pressure, or changes from low potassium.

17. 24-hour Holter monitor. A wearable ECG over a day or more detects intermittent rhythm spikes during hormone surges.

E) Imaging tests

18. Adrenal CT with non-contrast HU and washout. CT measures density (Hounsfield units). Malignant lesions are often dense (>10 HU), irregular, and show limited contrast washout; CT also stages spread.

19. Adrenal MRI with chemical-shift imaging. MRI helps separate benign lipid-rich adenomas from cancers; cancers enhance differently and may invade nearby vessels or organs.

20. Functional imaging (as appropriate).
    • MIBG scintigraphy targets catecholamine-producing tumors.
    • PET-CT (e.g., FDG) shows metabolically active cancers and metastases; DOTATATE PET can help with certain neuroendocrine tumors; FDOPA PET is useful for pheochromocytoma; 11C-metomidate PET can highlight cortical tissue in select centers.

Non-pharmacological treatments

Physiotherapy

1. Individualized aerobic training — Description: gentle walking or cycling 3–5 days/week, building up to 150 minutes/week as tolerated. Purpose: reduce fatigue and improve heart fitness. Mechanism: boosts oxygen delivery and mitochondrial efficiency. Benefits: better energy, mood, sleep, and treatment tolerance.

2. Progressive resistance exercise — Description: light weights or bands 2–3 days/week focusing on major muscle groups. Purpose: fight muscle loss from cancer and cortisol. Mechanism: stimulates muscle protein synthesis. Benefits: stronger body, better balance, easier daily tasks.

3. Inspiratory muscle training — Description: breathing through a handheld trainer 5–10 minutes twice daily. Purpose: support lungs around surgery/chemo. Mechanism: strengthens diaphragm and accessory muscles. Benefits: less shortness of breath and faster recovery.

4. Post-operative mobility program — Description: early walking, bed-to-chair transfers, and step goals after adrenalectomy. Purpose: prevent clots and lung issues. Mechanism: activates calf pump and improves ventilation. Benefits: fewer complications and shorter hospital stay.

5. Core and posture retraining — Description: targeted exercises for abdominal and back muscles. Purpose: reduce back pain from retroperitoneal surgery or tumor. Mechanism: stabilizes spine and improves load sharing. Benefits: less pain, better function.

6. Flexibility and stretching — Description: daily gentle stretches of hips, back, chest. Purpose: ease stiffness and scar tightness. Mechanism: lengthens muscle-tendon units and remodels collagen. Benefits: smoother movement and comfort.

7. Balance and gait training — Description: tandem stance, single-leg drills, safe obstacle walking. Purpose: prevent falls during weakness. Mechanism: retrains proprioception and reflexes. Benefits: safer mobility and confidence.

8. Pelvic and diaphragmatic breathing — Description: slow belly breathing several times daily. Purpose: calm nerves and reduce blood pressure surges. Mechanism: vagal activation lowers sympathetic tone. Benefits: fewer palpitations and anxiety episodes.

9. Scar management and soft-tissue mobilization — Description: guided massage and silicone sheeting after incision heals. Purpose: improve tissue glide. Mechanism: aligns collagen and reduces adhesions. Benefits: better range of motion and cosmetic outcome.

10. TENS for cancer-related pain — Description: low-risk skin electrodes used as advised. Purpose: reduce mild to moderate pain without extra pills. Mechanism: gate control and endorphin release. Benefits: less pain and lower opioid need.

11. Edema and venous thromboembolism prevention drills — Description: ankle pumps, calf squeezes, early ambulation. Purpose: lower clot risk after surgery. Mechanism: increases venous return. Benefits: fewer leg clots and swelling.

12. Neuromuscular re-education after prolonged steroids — Description: therapist-led functional movements. Purpose: reverse steroid myopathy. Mechanism: motor unit recruitment and hypertrophy. Benefits: stronger legs and easier stair climbing.

13. Heat/ice for focal discomfort — Description: brief, safe applications. Purpose: short-term pain relief. Mechanism: changes local blood flow and nerve signaling. Benefits: comfort to continue activity.

14. Fatigue-pacing and energy conservation — Description: plan, prioritize, pace tasks; use rests. Purpose: manage cancer-related fatigue. Mechanism: balances energy production and use. Benefits: more control over daily life.

15. Prehabilitation bundle — Description: 2–4 weeks of exercise, nutrition, and breathing practice before surgery. Purpose: raise fitness “reserve.” Mechanism: builds cardio-respiratory and muscular capacity. Benefits: faster post-op recovery and fewer complications.

Mind-Body, Gene, and Educational therapies

16. Mindfulness-based stress reduction — Description: daily 10–20 minutes of guided attention to breath and body. Purpose: reduce anxiety and pain perception. Mechanism: calms amygdala and lowers cortisol/adrenaline. Benefits: better sleep, mood, and blood pressure control.

17. Cognitive-behavioral therapy (CBT) — Description: brief weekly sessions to reframe unhelpful thoughts. Purpose: manage fear of recurrence and treatment stress. Mechanism: builds coping skills and problem-solving. Benefits: less distress and improved adherence.

18. Yoga or tai chi (gentle forms) — Description: low-impact sequences with breath control. Purpose: flexibility, balance, calm. Mechanism: combines movement with parasympathetic activation. Benefits: lower fatigue and improved quality of life.

19. Guided imagery and relaxation audio — Description: 10-minute recordings before scans or chemo. Purpose: ease anticipatory anxiety. Mechanism: visual-cortex engagement reduces limbic reactivity. Benefits: steadier vitals and comfort.

20. Sleep hygiene coaching — Description: regular schedule, dark cool room, screen curfew. Purpose: restore deep sleep. Mechanism: re-aligns circadian rhythm. Benefits: better energy and immune support.

21. Nutrition education for adrenal cancer — Description: consult with oncology dietitian. Purpose: maintain weight, muscle, and manage glucose. Mechanism: adequate protein/energy; fiber and micronutrients. Benefits: fewer complications and better tolerance to therapy.

22. Blood pressure self-monitoring training — Description: home BP cuff, log, and action plan. Purpose: catch surges in catecholamine or cortisol states. Mechanism: feedback loop supporting timely care. Benefits: safer day-to-day living.

23. Genetic counseling — Description: review family history and offer testing for TP53, SDHx, RET, etc. Purpose: clarify inherited risk. Mechanism: DNA testing guides surveillance and family planning. Benefits: early detection and informed relatives.

24. Precision-oncology education — Description: learning about tumor profiling, trials, and targets. Purpose: consider suitable studies when standard care is limited. Mechanism: matches biology to therapies. Benefits: more options and hope.

25. Medication and steroid-safety teaching — Description: how to take mitotane and stress-dose steroids; drug-interaction review. Purpose: prevent adrenal crisis and toxicity. Mechanism: patient knowledge and checklists. Benefits: safer treatment course.

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Drug treatments

Safety note: Doses vary by patient, kidney/liver function, and protocols. These are common reference ranges only; your oncology team decides exact plans.

1. Mitotane (adrenolytic; dose often 2–6 g/day in divided doses, titrated to serum level 14–20 mg/L; time continuous) — Purpose: core drug for ACC. Mechanism: destroys adrenal cortex cells and alters steroid synthesis. Side effects: nausea, dizziness, high lipids, neurocognitive changes; needs high-fat meals; many drug interactions; requires steroid replacement.

2. EDP regimen (Etoposide, Doxorubicin, Cisplatin) + Mitotane — Dose: Etoposide ~100 mg/m² d2–4, Doxorubicin ~40–50 mg/m² d1, Cisplatin ~40 mg/m² d1–2, q3–4 weeks with mitotane. Purpose: first-line for advanced ACC. Mechanism: multi-agent cytotoxic attack. Side effects: low blood counts, nausea, hair loss, neuropathy, kidney/heart risks (monitor).

3. Streptozocin + Mitotane (select cases) — Dose: protocols vary (e.g., 1 g/m² d1–5 q6wk). Purpose: alternative cytotoxic option. Mechanism: alkylates DNA in adrenocortical cells. Side effects: nausea, kidney toxicity, myelosuppression.

4. Pembrolizumab (PD-1 inhibitor; dose 200 mg IV q3wk or 400 mg q6wk) — Purpose: immunotherapy in mismatch-repair-deficient ACC or after chemo. Mechanism: releases T-cell brake. Side effects: immune-related thyroiditis, colitis, hepatitis; needs close monitoring.

5. Nivolumab ± Ipilimumab (PD-1 ± CTLA-4; dose per protocol) — Purpose: immunotherapy option in refractory disease. Mechanism: dual checkpoint blockade to boost anti-tumor immunity. Side effects: higher immune-toxicity when combined.

6. Cabozantinib (multi-kinase inhibitor; dose ~60 mg daily) — Purpose: off-label/selected refractory ACC or malignant pheo trials. Mechanism: blocks MET/VEGFR pathways to cut tumor blood supply. Side effects: hand–foot syndrome, diarrhea, hypertension.

7. Sunitinib or Sorafenib (TKIs; dose sunitinib 37.5–50 mg/day; sorafenib 400 mg BID) — Purpose: limited benefit, consider in selected cases or trials. Mechanism: VEGF and RAF pathway inhibition. Side effects: fatigue, skin reactions, BP rise.

8. Temozolomide (alkylating; dose ~150–200 mg/m² d1–5 q28d) — Purpose: salvage therapy in ACC or malignant pheo/paraganglioma (some responses in SDHB-mutated). Mechanism: DNA methylation causing tumor cell death. Side effects: myelosuppression, nausea.

9. Cisplatin/Carboplatin (doublets) — Dose: varies by regimen. Purpose: part of combination cytotoxic therapy for aggressive disease. Mechanism: cross-links DNA. Side effects: kidney/nerve/ear toxicity (cisplatin), marrow suppression (carboplatin).

10. Doxorubicin (anthracycline; dose capped by lifetime exposure) — Purpose: component of EDP; sometimes palliative single agent. Mechanism: intercalates DNA and generates free radicals. Side effects: cardiomyopathy risk, mucositis, nausea.

11. Etoposide — Purpose: EDP component. Mechanism: topoisomerase II inhibition. Side effects: myelosuppression, hair loss.

12. Cisplatin — Purpose: EDP component. Mechanism: DNA cross-linking. Side effects: nephrotoxicity, neuropathy, ototoxicity; strong anti-nausea plan needed.

13. Alpha-blockers (phenoxybenzamine or doxazosin) — Dose: titrated to BP/HR. Purpose: mandatory before surgery for malignant pheochromocytoma to prevent crises. Mechanism: blocks catecholamine effects. Side effects: dizziness, nasal stuffiness.

14. Beta-blockers (propranolol/metoprolol) — Use only after adequate alpha-blockade — Purpose: control tachycardia. Mechanism: slows heart rate. Side effects: fatigue; avoid before alpha-blockade.

15. Steroid replacement (hydrocortisone ± fludrocortisone) — Dose: physiologic replacement; stress-dose for illness/surgery. Purpose: counter adrenal suppression from mitotane or adrenalectomy. Mechanism: restores essential hormones. Side effects: if overdosed, weight gain, glucose rise.

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Dietary molecular supplements

1. Vitamin D3 — Dose: often 1000–2000 IU/day (or as guided by level). Function/mechanism: supports bone and immune function; mitigates steroid-induced bone loss. Note: check levels to avoid excess.

2. Calcium (with D) — Dose: ~1000–1200 mg/day from diet/supplement. Mechanism: bone mineral support during steroid therapy. Caution: separate from certain meds.

3. Omega-3 (EPA/DHA) — Dose: ~1–2 g/day combined EPA+DHA. Mechanism: anti-inflammatory; may help weight and appetite. Caution: bleeding risk with anticoagulants.

4. Protein supplement (whey/plant) — Dose: to meet ~1.2–1.5 g/kg/day total protein. Mechanism: supports muscle rebuilding.

5. Probiotics (evidence evolving) — Dose: per product. Mechanism: gut barrier and immune tone; may ease antibiotic-related diarrhea. Avoid when neutropenic unless approved.

6. Curcumin (turmeric extract) — Dose: commonly 500–1000 mg/day standardized extract. Mechanism: anti-inflammatory signaling; lab anti-tumor signals but human data limited. Caution: interacts with anticoagulants and some chemo.

7. Green tea extract (EGCG) — Dose: per label (e.g., 200–400 mg EGCG/day). Mechanism: antioxidant; modest evidence for fatigue. Caution: liver safety; avoid high doses with TKIs.

8. Selenium — Dose: 100–200 mcg/day if deficient. Mechanism: antioxidant enzyme cofactor; may support thyroid function if immunotherapy affects thyroid.

9. Magnesium — Dose: 200–400 mg/day as needed. Mechanism: helps low Mg from cisplatin; supports muscle and heart rhythm.

10. Multivitamin without iron (unless anemic) — Dose: daily. Mechanism: fills small gaps in intake during treatment.
    Mitotane is fat-soluble: taking medications with a meal that contains healthy fats can improve absorption (follow your team’s exact advice). Always check for interactions.

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Immunity-support / regenerative / stem-cell–related” medicines

1. Filgrastim (G-CSF) — Dose: daily injections around chemo cycles. Function: stimulates white blood cell recovery. Mechanism: expands neutrophil precursors.

2. Pegfilgrastim — Dose: single shot per chemo cycle. Function: long-acting neutrophil support.

3. Epoetin alfa/Darbepoetin — Dose: per hemoglobin thresholds. Function: treats chemo-related anemia. Mechanism: erythroid stimulation (use under strict oncology rules).

4. IVIG (intravenous immunoglobulin) for select cases — Function: passive immune support in severe hypogammaglobulinemia or autoimmune complications.

5. Thrombopoietin receptor agonists (eltrombopag/romiplostim) — Function: raise platelets in specific situations.

6. Clinical-trial cellular therapies — Function: experimental options (e.g., adoptive T-cell approaches) only within trials. Note: no standard stem-cell “drug” for ACC; avoid unproven clinics.

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Surgeries

1. Open adrenalectomy with en-bloc resection — Procedure: remove adrenal tumor and any invaded tissue (kidney, liver segment, diaphragm) with negative margins. Why: best chance of cure for localized ACC or malignant pheochromocytoma.

2. Laparoscopic/retroperitoneoscopic adrenalectomy — Procedure: minimally invasive removal for select small, localized tumors without invasion and for some malignant pheochromocytomas after alpha-blockade. Why: less pain and faster recovery when oncologically safe.

3. Regional lymph node dissection — Procedure: remove nearby nodes during adrenalectomy. Why: staging and may reduce local recurrence in ACC.

4. IVC thrombectomy — Procedure: remove tumor thrombus from the inferior vena cava when ACC extends into veins. Why: relieve obstruction and achieve complete resection.

5. Metastasectomy/debulking — Procedure: remove limited metastatic deposits or symptomatic bulk. Why: symptom relief, hormone control, and sometimes survival benefit in carefully selected patients.

(Ablation or stereotactic radiotherapy may be used for oligometastases in non-surgical candidates.)

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Preventions

Complete prevention is not always possible, but you can lower risks and catch problems early:

1. Genetic counseling/testing if family history fits.

2. Surveillance plans for known mutation carriers (regular labs and imaging).

3. Do not smoke; avoid secondhand smoke.

4. Maintain healthy weight and active lifestyle.

5. Balanced diet rich in whole foods and fiber.

6. Control blood pressure and blood sugar.

7. Limit alcohol.

8. Protect from unnecessary radiation exposure.

9. Know your medicines (some interact with mitotane and BP).

10. Keep all follow-up visits for early detection of recurrence.

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When to see doctors urgently or promptly

• Severe, sudden headache, pounding heartbeat, and sweating spells.

• Very high blood pressure or chest pain.

• Weakness with vomiting, dizziness, or fever after adrenal surgery or while on mitotane (possible adrenal crisis).

• Rapid weight gain, swelling, or skin changes suggesting cortisol excess.

• Any new severe pain, especially in bones or abdomen.

• Unexplained low potassium, repeated.

• New neuro symptoms (confusion, severe fatigue).

• Before starting any new supplement or medication (interaction risk).

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Foods to favor and to avoid

What to eat

1. High-protein foods (fish, eggs, legumes) for muscle.

2. Healthy fats (olive oil, avocado, nuts) — helpful with mitotane absorption.

3. Whole grains and fiber for glucose control.

4. Colorful vegetables and fruits (antioxidant variety).

5. Calcium-rich foods (dairy, fortified plant milks) with vitamin D.

What to avoid or limit

1. Sugary drinks and ultra-processed snacks (glucose spikes).
2. Excess salt if hypertensive/aldosterone excess.
3. Alcohol (limit; interacts with meds).
4. Grapefruit/Seville orange (CYP3A4 interactions with some drugs).
5. Tyramine-rich foods and stimulants (aged cheeses, certain cured meats, energy drinks) if pheochromocytoma is suspected/managed.

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Frequently asked questions

1. Is adrenal cancer curable?
   Sometimes. Small, localized tumors completely removed have the best chance. Advanced disease is often treatable but not always curable.

2. What is the main drug for ACC?
   Mitotane, often combined with chemotherapy such as EDP. Levels and side effects must be closely monitored.

3. Will I need steroids?
   Many patients do, especially with mitotane or after adrenal removal, because natural cortisol can become too low.

4. Can immunotherapy help?
   It may help some patients, especially if the tumor has mismatch-repair defects or high mutation burden. Your team will test for this.

5. Do all adrenal tumors make hormones?
   No. Some are “non-functioning.” Others overproduce cortisol, aldosterone, androgens, estrogen, or catecholamines.

6. Is biopsy always needed?
   Not always. If imaging and hormones strongly suggest pheochromocytoma, biopsy is avoided until it is safely blocked; many ACCs go directly to surgery if resectable.

7. How often will I need scans?
   Typically every 3–6 months for the first years, then less often, depending on stage and treatment.

8. Can I exercise during treatment?
   Yes, with a tailored plan. Exercise often reduces fatigue and improves outcomes.

9. What are common side effects of mitotane?
   Nausea, dizziness, high cholesterol, and thinking/memory changes. It interacts with many drugs. Levels are checked often.

10. Does diet fight the cancer?
    Diet supports strength, weight, and treatment tolerance. No food cures cancer, but good nutrition helps you do better.

11. Should family members be tested?
    If a hereditary syndrome is suspected, genetic counseling for relatives is recommended.

12. What is malignant pheochromocytoma?
    A catecholamine-producing adrenal tumor that has spread or shows malignant behavior. It needs alpha-blockade and specialized care.

13. Can adrenal cancer come back?
    Yes. Regular follow-up and early treatment of recurrence are important.

14. Are clinical trials important?
    Yes. Because adrenal cancers are rare, trials can provide access to promising therapies and improve future care.

15. What specialist should I see?
    A multidisciplinary team: endocrine surgeon, medical oncologist with adrenal expertise, endocrinologist, genetic counselor, and supportive-care clinicians.

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 09, 2025.

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