Malignant Neoplasm of the Adrenal Gland

A malignant neoplasm of the adrenal gland means cancer that starts in one of the two small glands sitting on top of your kidneys. Each adrenal gland has two main parts. The cortex (outer layer) makes steroid hormones such as cortisol, aldosterone, and androgens (sex hormones). The medulla (inner core) makes catecholamines such as adrenaline (epinephrine) and noradrenaline (norepinephrine). When cells in either part grow in an uncontrolled way and invade nearby tissues or spread (metastasize) to other organs, the growth is called malignant. Some adrenal cancers produce excess hormones, causing clear body changes; others are non-functioning and grow quietly until they become large. Adrenal cancer is rare but can be serious because it may spread early, press on nearby organs, or cause dangerous hormone surges. Early recognition and careful testing are very important because treatment plans depend on the tumor’s origin (cortex or medulla), hormone output, and stage.

A malignant neoplasm of the adrenal gland is a cancer that starts in one of the two layers of your adrenal glands, which sit above the kidneys. Tumors from the outer layer (cortex) are called adrenocortical carcinoma (ACC) and may make too much steroid hormone (cortisol, aldosterone, or androgens). Tumors from the inner layer (medulla) are pheochromocytomas, and when malignant (metastasized), they’re often grouped with paragangliomas (PPGLs). These can release high levels of adrenaline-like chemicals (catecholamines), causing dangerous blood-pressure spikes. The main goal of care is complete removal when possible, careful control of hormones, and—if it has spread—medicines and targeted treatments to slow or control the disease. Complete “R0” removal with clear margins gives the best chance of long-term control in ACC. The LancetAnnals of Oncology

Other names

People may call this illness adrenal cancer, malignant adrenal tumor, adrenal gland carcinoma, adrenocortical carcinoma (ACC) when it starts in the cortex, or malignant pheochromocytoma when it starts in the medulla. In children, adrenal cancers may be grouped with adrenal cortical tumors or, less often, neuroblastoma if arising from nerve-like cells. Medical records may use terms like malignant neoplasm of adrenal gland, adrenal cortical cancer, adrenal medullary cancer, functional adrenal carcinoma (hormone-secreting), nonfunctional adrenal carcinoma, or simply adrenal malignancy.

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Types

1) By cell of origin

• Adrenocortical carcinoma (ACC): A malignant tumor of the adrenal cortex. It may make too much cortisol (Cushing’s syndrome), aldosterone (leading to high blood pressure and low potassium), or androgens/estrogens (causing changes in hair, voice, periods, breast tissue, or growth in children). ACC tends to be aggressive but is potentially curable if caught early and removed completely.

• Malignant pheochromocytoma (adrenal medulla): A cancer from chromaffin cells that release catecholamines. It can cause sudden high blood pressure, pounding heart, headaches, sweating, palpitations, pallor, and anxiety. Some pheochromocytomas are benign; “malignant” means proven spread or clear invasion.

• Other primary adrenal cancers (rare): These include primary adrenal lymphoma, sarcoma, or very rare mixed tumors.

• Secondary (metastatic) tumors to the adrenal: Many cancers from other organs (lung, kidney, melanoma, colon, etc.) can spread to the adrenal. These are not primary adrenal cancers, but they do cause an adrenal malignant neoplasm in reports.

2) By hormone function

• Functioning tumors: Overproduce one or more hormones (cortisol, aldosterone, androgens, estrogens, or catecholamines). Signs often point to the diagnosis.

• Non-functioning tumors: Do not make excess hormones. They may grow silently and be found incidentally on scans or when large enough to press on organs.

3) By stage and behavior

• Localized: Limited to the adrenal gland, no spread.

• Locally advanced: Invades nearby structures (kidney, liver, major veins such as the inferior vena cava).

• Metastatic: Spreads to distant organs (lungs, liver, bones, lymph nodes).

• Recurrent: Returns after treatment.

4) By grade/biology

• Pathologists may report Ki-67 index (how quickly cells divide) or use systems like Weiss/Helsinki criteria in ACC to estimate aggressiveness. Higher grades usually behave more aggressively.

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Causes and risk factors

In cancer, “cause” often means risk factor or genetic driver. Many patients have no clear risk. The items below describe known or suspected contributors.

1. Li-Fraumeni syndrome (TP53 mutation): An inherited change in the TP53 “guardian” gene greatly raises the risk of ACC, especially in children and in certain families. It allows damaged cells to keep dividing instead of dying.

2. Beckwith–Wiedemann spectrum (IGF2 dysregulation): Children with this overgrowth condition have more growth signals (like IGF2), making adrenal cortical tumors, including ACC, more likely.

3. Multiple endocrine neoplasia type 2 (RET mutations): Strongly linked to pheochromocytomas; a small portion can be malignant. The RET change drives hormone-producing medullary cells to grow.

4. Von Hippel–Lindau (VHL) disease: This inherited condition alters oxygen-sensing pathways and is linked to pheochromocytomas and paragangliomas; some become malignant.

5. Neurofibromatosis type 1 (NF1): Changes in the NF1 gene increase nerve-related tumors and can include pheochromocytoma risk.

6. Succinate dehydrogenase (SDHB/SDHD/SDHC) mutations: These changes tilt cell metabolism and oxygen sensing, raising the risk of paraganglioma/pheochromocytoma, and SDHB in particular is linked to higher malignancy risk.

7. MAX and TMEM127 gene variants: Less common inherited changes that predispose to adrenal medulla tumors; malignancy can occur.

8. Mismatch repair gene changes (Lynch spectrum): Rare families with MSH2/MLH1 defects have reported ACC; defective DNA repair lets mutations build up.

9. Southern Brazil TP53 founder variant (R337H): A regional inherited TP53 variant leads to higher childhood ACC rates.

10. Family history of adrenal tumors: Even without a named syndrome, families with multiple cases hint at shared genetic risks.

11. Prior radiation exposure (childhood or therapeutic): Radiation can damage DNA; a small rise in risk for adrenal or medulla-related tumors is reported in some survivor groups.

12. Congenital adrenal hyperplasia (CAH): Long-term stimulation and nodular growth of the cortex may increase the chance of neoplasia in some patients.

13. Age extremes: ACC peaks in young children and again in adults 40–60. Pheochromocytoma often appears in mid-adulthood, earlier with inherited genes.

14. Female sex (for ACC): Slight female predominance is seen in many ACC series, though reasons are not fully clear.

15. Chronic hypoxia states (for paraganglioma pathways): Low-oxygen signaling can drive HIF pathways that favor medulla-type tumor growth; high-altitude residence and cyanotic heart disease are examples.

16. Obesity and metabolic syndrome: These conditions alter hormones and inflammation; they are linked with adrenal nodules and possibly tumor progression.

17. Environmental carcinogens (general): Long-term exposure to DNA-damaging chemicals may contribute, though specific agents for adrenal cancer are not firmly proven.

18. Somatic tumor mutations (non-inherited): In ACC, frequent changes involve TP53, CTNNB1 (β-catenin), ZNRF3, MEN1, and others; in pheochromocytoma, RET, VHL, NF1, SDHx, MAX, TMEM127 pathways are key.

19. Immunologic dysregulation (rare): Primary adrenal lymphoma arises from lymphoid cells within the gland; mechanisms differ from ACC/pheochromocytoma but still cause adrenal malignancy.

20. Unknown/sporadic factors: Most patients have no clear trigger; random DNA errors over time can start cancer in adrenal cells.

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Symptoms and signs

1. Abdominal or flank pain: A growing mass stretches the capsule of the adrenal or presses on nearby organs.

2. Abdominal fullness or a palpable lump: Large tumors push the stomach, liver, or kidney and can be felt or seen on imaging.

3. Unintentional weight loss and fatigue: Cancer cells use energy and cause inflammation; appetite may fall.

4. Cushing-like features (from cortisol excess): Round face, easy bruising, purple stretch marks, thin skin, central weight gain, mood changes, and weak muscles occur when the tumor makes too much cortisol.

5. High blood pressure (various pathways): Cortisol, aldosterone, or catecholamines can all raise blood pressure; pheochromocytoma often causes sudden spikes.

6. Low potassium symptoms (aldosterone excess): Muscle cramps, weakness, or palpitations happen when aldosterone makes kidneys waste potassium.

7. Virilization in women (androgen excess): New coarse hair, acne, deeper voice, menstrual changes, and clitoral enlargement suggest androgen-secreting ACC.

8. Feminization in men (estrogen excess): Breast enlargement (gynecomastia), reduced libido, or impotence may point to estrogen-secreting ACC.

9. Rapid growth and early puberty in children: Excess androgens or estrogens cause fast height gain, body hair, acne, or early periods.

10. Headaches, palpitations, sweating spells, tremor (pheochromocytoma): Surges of catecholamines cause the classic triad: headache, sweating, and palpitations, often with pallor and anxiety.

11. Anxiety or panic-like attacks: Sudden hormone surges can mimic panic episodes; attacks may be triggered by stress, surgery, or certain drugs.

12. Back pain: Tumor pressure on muscles or spine structures can cause dull or sharp pain.

13. Nausea, vomiting, or early satiety: A large adrenal mass compresses the stomach and gut.

14. Bone pain or fractures (advanced disease): Spread to bone or severe cortisol excess (which thins bone) causes aches or breaks.

15. Swelling of legs or abdominal veins (large right-sided tumors): Invasion into major veins (renal vein or inferior vena cava) can block blood flow.

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Diagnostic tests

A) Physical examination

1. General inspection for hormone effects: Your clinician looks for Cushing features (bruises, stretch marks, central fat), virilization or feminization signs, acne, and skin thinning. These visible clues suggest a functioning adrenal tumor.

2. Vital signs and repeated blood pressure checks: Sustained or paroxysmal hypertension with rapid heart rate hints at pheochromocytoma. Very high pressures during spells are red flags.

3. Abdominal and back examination: Gentle palpation may find a mass or tender area. The clinician also checks for liver enlargement (possible spread) and listens for bruits over big veins if invasion is suspected.

B) Manual/bedside tests

4. Orthostatic blood pressure and pulse: Measurements lying, then standing. A large rise in blood pressure or heart rate swings can occur with catecholamine surges or volume changes.

5. Manual muscle strength testing: Proximal muscle weakness (trouble rising from a chair) supports cortisol excess from a functioning ACC.

6. Clinical scoring of hirsutism and sexual development: Tools like the Ferriman–Gallwey score or Tanner staging in children help quantify androgen/estrogen effects from the tumor.

C) Laboratory and pathological tests

7. Plasma free metanephrines (or 24-hour urine fractionated metanephrines): The best screening for pheochromocytoma. Metanephrines are stable breakdown products of catecholamines and are typically high when the tumor secretes adrenaline/noradrenaline.

8. 24-hour urinary catecholamines and VMA: A complementary test that captures hormone output over a day, useful if blood tests are borderline.

9. Overnight 1-mg dexamethasone suppression test: You take dexamethasone at night; a healthy adrenal suppresses cortisol by morning. Failure to suppress suggests Cushing syndrome from a functioning ACC.

10. Late-night salivary cortisol (and/or 24-hour urinary free cortisol): Cortisol should be low late at night; persistently high levels support autonomous cortisol production.

11. Plasma ACTH: Low ACTH with high cortisol points to an adrenal source (ACTH-independent Cushing), helping separate adrenal tumors from pituitary disease.

12. Aldosterone-to-renin ratio (if hypertension/hypokalemia): A very high ratio suggests autonomous aldosterone production. In cancer this is uncommon, but it is checked when the clinical picture fits.

13. Sex steroid profile (DHEA-S, androstenedione, testosterone; estradiol in men): Marked elevation suggests a steroid-secreting ACC, guiding both diagnosis and follow-up.

14. Pathology on tumor tissue with proliferation index (Ki-67) and ACC scoring (Weiss/Helsinki): After surgical removal (or very selective biopsy in special cases), the pathologist confirms malignancy, estimates aggressiveness, and looks for margins and vascular invasion.

Important safety note: Fine-needle biopsy is generally avoided when pheochromocytoma is possible because puncturing a catecholamine-secreting tumor can trigger a dangerous surge. Biopsy is reserved for situations like suspected metastasis from another cancer after biochemical exclusion of pheochromocytoma.

15. Genetic testing for hereditary syndromes (RET, VHL, NF1, SDHx, TP53, MAX, TMEM127, etc.): Offered when age is young, there is bilateral disease, a family history, or multiple tumors. Results guide family counseling and long-term screening.

16. General blood tests (electrolytes, glucose/HbA1c, kidney and liver function): These measure complications (low potassium, high glucose) and provide a baseline before any treatment.

D) Electrodiagnostic and monitoring tests

17. Electrocardiogram (ECG): Looks for fast rhythms, ischemic changes, or QT problems from catecholamine excess or electrolyte shifts (e.g., low potassium). It helps risk-stratify patients with palpitations and hypertension.

18. Ambulatory ECG/Holter or telemetry during spells: Captures episodic tachycardia or arrhythmias that match catecholamine surges, supporting the diagnosis of a functioning medulla tumor.

E) Imaging tests

19. Adrenal protocol CT scan with contrast and washout: The main imaging test for adrenal masses. It measures size, shape, density, contrast washout, and local invasion. High density, irregular edges, delayed washout, tumor thrombus, or very large size suggest malignancy.

20. MRI of the adrenals (chemical-shift and vascular sequences) and functional scans (when needed): MRI distinguishes lipid-rich benign adenomas from cancers, shows vascular invasion, and is valuable if CT contrast is not suitable. Functional imaging helps:

• ^123I-MIBG or ^68Ga-DOTATATE PET/CT for pheochromocytoma/paraganglioma mapping.

• ^18F-FDG PET/CT for ACC staging, detecting active metastases, and surgical planning.

Treatment overview

• Surgery aiming at R0 (negative margins) is the main curative option for localized ACC and is done as an open, en-bloc adrenalectomy in most suspected ACC. Minimally invasive approaches are highly selective due to higher recurrence signals in ACC. PMCSpringerLink

• Mitotane is the only drug approved specifically for ACC; used adjuvantly in high-risk cases and for advanced disease. The EDP-M regimen (etoposide, doxorubicin, cisplatin plus mitotane) is the first-line standard in metastatic ACC per the randomized FIRM-ACT trial and subsequent guideline updates. New England Journal of MedicinePMCScienceDirect

• For malignant pheochromocytoma/paraganglioma, options include CVD chemotherapy (cyclophosphamide, vincristine, dacarbazine), temozolomide (especially in SDHB), tyrosine kinase inhibitors (e.g., sunitinib), and ^131I-MIBG therapy for MIBG-avid disease; PRRT can be considered for somatostatin receptor–positive tumors. Oxford AcademicEJCancerScienceDirect+1

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Non-pharmacological treatments

Physiotherapy & cancer-rehab 

1. Energy-conservation training — Description: Learn to pace activities, plan rest, and cluster tasks. Purpose: Reduce cancer-related fatigue. How: Balances activity with recovery to prevent “crash-and-burn.” Benefits: More stable daily energy, better function.

2. Graded aerobic conditioning — Description: Walking or cycling 20–30 min most days, scaled to capacity. Purpose: Improve stamina and heart health. How: Gradual VO₂ and mitochondrial gains. Benefits: Less fatigue, better BP/sugar control (important in cortisol excess).

3. Progressive resistance training — Description: 2–3 sessions/week for major muscle groups. Purpose: Reverse muscle loss from high cortisol or chemo. How: Stimulates muscle protein synthesis. Benefits: Strength, balance, bone support.

4. Core and posture therapy — Description: Target trunk stabilizers and spinal alignment. Purpose: Ease back pain from mass effect/deconditioning. How: Improves load sharing and mechanics. Benefits: Less pain, safer movement.

5. Flexibility & mobility work — Description: Gentle stretching/yoga-style mobility. Purpose: Reduce stiffness from steroids or inactivity. How: Increases range of motion and tissue glide. Benefits: Comfort, movement confidence.

6. Balance & gait training — Description: Tandem stands, step drills. Purpose: Prevent falls (steroid myopathy, neuropathy risk). How: Retrains vestibular/proprioceptive systems. Benefits: Safety, independence.

7. Breathing retraining — Description: Diaphragmatic and paced breathing. Purpose: Manage anxiety/pheo spells; aid pain control. How: Lowers sympathetic drive. Benefits: Calmer episodes, better exercise tolerance.

8. Pelvic & trunk stability for cough/strain — Description: Safe bracing strategies. Purpose: Protect incision after adrenalectomy. How: Activates deep stabilizers. Benefits: Fewer post-op discomfort flares.

9. Manual therapy (gentle) — Description: Soft-tissue techniques around non-tumor areas. Purpose: Ease post-op tension. How: Modulates pain pathways. Benefits: Comfort, sleep.

10. Lymphedema-aware care — Description: Edema surveillance, compression if indicated after extended lymphadenectomy. Purpose: Swelling control. How: External pressure + exercise. Benefits: Less heaviness, infection risk.

11. Scar and incision care education — Description: Mobilization after healing, sunscreen, desensitization. Purpose: Reduce adhesions, pain. How: Tissue remodeling cues. Benefits: Softer scar, easier motion.

12. Return-to-work conditioning — Description: Work-specific tasks. Purpose: Safe re-entry to job demands. How: Gradual load progression. Benefits: Confidence, productivity.

13. Sleep optimization program — Description: Consistent schedule, light control, CBT-I tips. Purpose: Restore restorative sleep disrupted by steroids/stress. How: Circadian alignment. Benefits: Energy, mood, immunity support.

14. Therapeutic heat/cold (local) — Description: Brief heat for stiffness, cold for acute soreness (not over tumor field pre-op). Purpose: Symptom relief. How: Alters nociception and circulation. Benefits: Less pain, easier therapy.

15. Safe BP-rise management plan — Description: With physio present: warm-up/cool-down, avoid Valsalva. Purpose: Reduce pheo-related spikes during activity. How: Minimizes sympathetic surges. Benefits: Safer sessions.

Mind–body, “gene-informed,” and educational (

1. Psycho-oncology counseling — Description: Brief CBT/ACT to handle fear and uncertainty. Purpose: Reduce distress. How: Skills for thoughts/feelings. Benefits: Adherence, quality of life.
2. Guided relaxation & mindfulness — Description: Daily 10–15 min practice. Purpose: Lower sympathetic tone. How: Parasympathetic activation. Benefits: Fewer palpitations, better sleep.
3. Patient education for adrenal hormones — Description: Simple modules on cortisol, aldosterone, catecholamines. Purpose: Recognize warning signs. How: Teaches patterns and triggers. Benefits: Early help-seeking, safer self-care.
4. Crisis card & home BP toolkit — Description: Wallet card with diagnosis, meds, emergency steps; validated BP cuff. Purpose: Safer episodes. How: Standardized response. Benefits: Faster care, reduced ER risk.
5. Family genetic counseling — Description: Offer testing for TP53/MMR/RET/VHL/NF1/SDHx when indicated. Purpose: Detect heritable risk; guide screening. How: Germline testing + cascade testing. Benefits: Early detection in relatives. PubMedJNCCN
6. Trigger-avoidance plan (pheo) — Description: Limit stimulants (caffeine), decongestants; manage stressors. Purpose: Fewer paroxysms. How: Avoid adrenergic triggers. Benefits: Safer day-to-day.
7. Pre-op education for alpha-blockade — Description: Teach high-salt/hydration as directed after alpha-blocker start for pheo surgery prep. Purpose: Prevent complications. How: Maintains intravascular volume. Benefits: Smoother surgery.
8. Dietitian-led metabolic plan — Description: Tailored protein, calcium, vitamin D, potassium needs; diabetes support. Purpose: Counter steroid and catecholamine effects. How: Nutrient timing and composition. Benefits: Better labs, bone, energy.
9. Return-to-activity “red-flag” teaching — Description: Identify symptoms that require medical review. Purpose: Safety net. How: Simple rules. Benefits: Early detection.
10. Peer support / survivorship groups — Description: Connect with others. Purpose: Reduce isolation. How: Shared strategies. Benefits: Coping, adherence.

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Drug treatments

Important: Doses are examples for adults and must be individualized by your oncology/endocrine team.

1. Mitotane (adrenolytic) • Dose: titrated to serum levels (often 2–6 g/day divided) • Purpose: cornerstone for ACC (adjuvant/high-risk or metastatic) • Mechanism: selective adrenal cortical cytotoxicity; hormone synthesis inhibition • Side effects: GI upset, neurotoxicity, adrenal insufficiency (needs steroid replacement), drug interactions. New England Journal of MedicinePMC

2. Etoposide (topoisomerase II inhibitor; part of EDP-M) • Typical: days 2–4 in 28-day cycles • Purpose: metastatic ACC combo • Mechanism: DNA strand break accumulation • Side effects: myelosuppression, alopecia, mucositis. PMC

3. Doxorubicin (anthracycline; EDP-M) • Purpose: ACC regimen • Mechanism: intercalation/free radicals • Side effects: cardiotoxicity, myelosuppression; lifetime dose monitoring. PMC

4. Cisplatin (platinum; EDP-M) • Purpose: ACC regimen • Mechanism: DNA crosslinks • Side effects: nephrotoxicity, nausea, neuropathy; hydration needed. PMC

5. Streptozocin (alkylator) • Dose: per protocol with mitotane (FIRM-ACT comparator) • Purpose: alternative in ACC • Side effects: nephrotoxicity, nausea. PMC

6. Temozolomide (alkylator) • Dose: e.g., 150–200 mg/m² d1–5 q28d • Purpose: metastatic PPGL (esp. SDHB) • Mechanism: DNA methylation (O6-guanine) • Side effects: cytopenias, fatigue, nausea. Oxford Academic

7. CVD regimen (cyclophosphamide + vincristine + dacarbazine) • Purpose: metastatic PPGL • Mechanism: multi-agent cytotoxic • Side effects: cytopenias, neuropathy (vincristine), nausea. Oxford Academic

8. Sunitinib (multi-target TKI) • Dose: common 37.5–50 mg daily schedules • Purpose: progressive metastatic PPGL • Mechanism: anti-angiogenic (VEGFR, etc.) • Side effects: hypertension, fatigue, hand-foot syndrome. EJCancerScienceDirect

9. ^131I-MIBG (iobenguane) therapy (radiopharmaceutical) • Purpose: MIBG-avid PPGL • Mechanism: norepinephrine analog taken up by tumor, delivers targeted radiation • Side effects: cytopenias, nausea; radiation safety steps. ScienceDirect

10. Somatostatin analogs (octreotide/lanreotide) • Purpose: symptom control and potential disease stabilization in receptor-positive PPGL • Mechanism: binds somatostatin receptors • Side effects: GI upset, gallstones, glucose changes. PMC

11. PRRT (e.g., ^177Lu-DOTATATE) • Purpose: receptor-positive PPGL with progression • Mechanism: peptide-targeted radiation • Side effects: nausea, cytopenias, rare renal effects. PMC

12. Alpha-blockers (phenoxybenzamine or doxazosin) • Purpose: pre-op and symptomatic BP control in pheo • Mechanism: alpha-adrenergic blockade • Side effects: orthostasis, nasal congestion; start before beta-blocker. PubMed

13. Beta-blockers (e.g., propranolol, metoprolol) • Purpose: rate control after adequate alpha-blockade • Mechanism: beta-adrenergic blockade • Side effects: bradycardia, fatigue; never start before alpha-blockade in pheo. PubMed

14. Metyrosine • Purpose: reduces catecholamine synthesis in refractory pheo • Mechanism: inhibits tyrosine hydroxylase • Side effects: sedation, depression, crystalluria; hydration advised. PubMed

15. Steroidogenesis blockers (ketoconazole, metyrapone; mifepristone as receptor antagonist) • Purpose: control hypercortisolism when needed alongside ACC care • Mechanism: block cortisol synthesis or action • Side effects: liver toxicity (ketoconazole), BP/electrolyte shifts (metyrapone), endometrial changes/hypokalemia (mifepristone). PMC

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Dietary molecular supplements

These can support strength, bones, and metabolism; none cures cancer. Check for interactions, especially because mitotane and many chemo drugs interact with supplements.

1. Vitamin D3 (e.g., 800–2000 IU/day; higher if deficient) — supports bone health in cortisol excess; aids muscle function.

2. Calcium (1000–1200 mg/day total intake) — protects bones if cortisol is high or if on steroids; split doses.

3. Protein (whey/plant) 1.2–1.5 g/kg/day — preserves muscle during treatment; space across meals.

4. Omega-3 fatty acids (EPA/DHA ~1–2 g/day) — may help inflammation and cancer-related weight loss; watch bleeding risk.

5. Magnesium (200–400 mg/day) — supports muscle/nerve, may help if cisplatin lowers magnesium; monitor labs.

6. Potassium-rich foods/supplement only if needed — corrects aldosterone-related losses; monitor with your team.

7. CoQ10 (100–200 mg/day) — may improve fatigue in some; interaction potential is low but discuss first.

8. Selenium (55–200 mcg/day) — antioxidant support; avoid high doses.

9. Curcumin standardized extract — anti-inflammatory potential; possible drug interactions (CYP); discuss before use.

10. Probiotics (strain-specific, daily) — may reduce antibiotic-related GI effects; choose medically reviewed products.

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Immunity booster / regenerative / stem-cell–related” drugs

There are no approved stem-cell drugs for adrenal cancers. “Regenerative” therapies are investigational. These items are supportive or immunotherapeutic and used under oncology guidance.

1. Filgrastim (G-CSF) — Dose: weight-based daily after chemo • Function: raises neutrophils to prevent infections • Mechanism: stimulates marrow granulopoiesis.

2. Pegfilgrastim (long-acting G-CSF) — Dose: single injection per chemo cycle • Function/Mechanism: as above, sustained.

3. Sargramostim (GM-CSF) — Function: broader myeloid stimulation • Mechanism: GM-CSF receptor activation.

4. Vaccinations (influenza, COVID-19, pneumococcal as indicated) — Function: reduces infection risk • Mechanism: adaptive immune priming (schedule per oncology).

5. Immune checkpoint inhibitors (e.g., pembrolizumab, nivolumab±ipilimumab in select settings) — Function: unleash anti-tumor immunity in a subset • Mechanism: PD-1/PD-L1/CTLA-4 blockade • Note: evidence in ACC/PPGL is evolving; immune adverse events possible.

6. Erythropoiesis-stimulating agents (when appropriate) — Function: treat symptomatic chemo-related anemia • Mechanism: EPO receptor activation (restricted use by guidelines).

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Surgeries

1. Open en-bloc adrenalectomy (standard for suspected ACC) — removes adrenal tumor with surrounding fat ± involved structures, aiming for R0 margins. Why: best chance of cure/local control. PMC

2. Adrenalectomy with vena cava thrombectomy (if tumor thrombus) — specialized, removes tumor extension into major veins. Why: clear margins and restore blood flow. PMC

3. Selective lymphadenectomy — removes regional nodes when involved/suspected. Why: staging and local control in ACC. PMC

4. Minimally invasive adrenalectomy (carefully selected small, well-staged tumors) — Why: faster recovery when oncologically safe; not routine for overt ACC due to recurrence risks. SpringerLink

5. Metastasectomy / cytoreductive surgery (selected patients) — removes limited metastases (lung/liver) after MDT review. Why: symptom control, potential survival benefit in highly selected scenarios. PMC

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Prevention tips

1. Know your family risk — seek genetic counseling/testing if you or close relatives have ACC or PPGL. PubMed

2. Regular follow-up for known adrenal masses per imaging/lab algorithms. PMC

3. Healthy weight, balanced diet, regular exercise — supports overall cancer resilience.

4. Quit smoking; avoid vaping — general cancer risk reduction.

5. Limit alcohol — reduces BP and liver strain.

6. Avoid stimulant decongestants (e.g., pseudoephedrine) if you have or are at risk for pheo. PubMed

7. Manage blood pressure and diabetes — lowers complications.

8. Radiation prudence — use imaging wisely; follow guidelines.

9. Bone health habits — calcium/vitamin D, resistance exercise if cortisol is/was high.

10. Adhere to surveillance plans after treatment (imaging, hormone labs).

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When to see a doctor

• Severe headache with pounding heartbeat, sweating, and very high BP (possible pheo crisis) — urgent care now.

• Sudden chest pain, shortness of breath, fainting, or palpitations — emergency.

• New or rapidly worsening high blood pressure, especially in episodes.

• Cushingoid changes (rapid weight gain, easy bruising, purple stretch marks), new virilization, or unexplained low potassium.

• Abdominal pain/fullness that persists or worsens.

• After treatment: fever ≥38°C, uncontrolled vomiting/diarrhea, bleeding, or signs of adrenal insufficiency (severe fatigue, dizziness, nausea, low BP).

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What to eat” and “what to avoid

Eat more of:

1. Lean proteins (fish, poultry, legumes) for muscle repair.

2. High-fiber whole grains for steady glucose.

3. Fruits/vegetables of many colors for micronutrients.

4. Calcium & vitamin D sources (dairy or fortified alternatives) for bones.

5. Potassium-rich foods (if potassium is low and your doctor agrees): leafy greens, bananas, beans.

Limit/avoid:

1. Caffeine and energy drinks if pheo or BP spikes.
2. Decongestants and stimulant supplements (check labels).
3. Very salty foods unless you’re in the brief pre-op alpha-blockade phase (where extra salt is sometimes instructed).
4. Alcohol excess (raises BP and sugar).
5. Ultra-processed, high-sugar foods (worsen glucose and weight).

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Frequently asked questions

1. Is adrenal cancer curable?
   If caught early and removed completely (R0), ACC can be controlled long-term; malignant PPGL outcomes vary. Follow-up is essential. Annals of Oncology

2. What is the first treatment for localized ACC?
   Open adrenalectomy aiming for clear margins. PMC

3. Do all adrenal tumors make hormones?
   No. Many are non-functioning; others overproduce cortisol, aldosterone, or androgens (ACC) or catecholamines (pheo/para). PubMed

4. How do doctors screen for pheochromocytoma?
   Plasma free metanephrines or 24-hour urine metanephrines. PubMed

5. What scans are used?
   CT/MRI for anatomy; PET for spread; functional scans like MIBG or DOTATATE in PPGL. PMCAmerican Cancer SocietyScienceDirect

6. What chemo is standard for metastatic ACC?
   EDP-M (etoposide, doxorubicin, cisplatin + mitotane). PMCScienceDirect

7. Is mitotane always needed?
   Not always, but it’s key in many ACC settings; oncologists titrate to target blood levels and add steroid replacement as needed. New England Journal of Medicine

8. What about targeted drugs for PPGL?
   Sunitinib and others can help in selected cases; decisions are individualized. EJCancer

9. Can radiation treat these tumors?
   Yes, for palliation or targeted (^131I-MIBG in MIBG-avid PPGL; PRRT in receptor-positive disease). ScienceDirectPMC

10. Should I get genetic testing?
    Often yes—ACC and PPGL have meaningful hereditary links; your team will guide testing. PubMedJNCCN

11. Is laparoscopic adrenalectomy okay for ACC?
    Usually open is preferred for suspected ACC due to oncologic concerns; minimally invasive is reserved for carefully selected cases. SpringerLink

12. How often will I be scanned after surgery?
    Typically every few months initially, then less often if stable; exact schedule varies by tumor type and stage. NCCN

13. Can diet cure the cancer?
    No. Diet supports strength and tolerance of therapy; it does not replace medical/surgical treatment.

14. What if I have severe headaches and high BP suddenly?
    This can be a pheo crisis—seek emergency care immediately.

15. Will I need hormones after mitotane or surgery?
    Possibly. Mitotane and adrenalectomy can cause adrenal insufficiency requiring steroid replacement; your team will monitor you.

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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