Malignant Adrenal Gland Tumor

A malignant adrenal gland tumor is a cancer that starts in one of the two small adrenal glands that sit above the kidneys. The adrenal cortex (the outer layer) makes steroid hormones such as cortisol, aldosterone, and sex steroids; cancers from this layer are called adrenocortical carcinoma (ACC). The adrenal medulla (the inner core) makes catecholamines (adrenaline-like hormones); tumors here are pheochromocytomas, which are called malignant when they spread to places where chromaffin tissue does not normally exist. Malignant adrenal tumors may overproduce hormones, grow silently and be found on a scan done for another reason, or present late with pain, weight loss, and spread to other organs. Diagnosis relies on careful hormone testing and high-quality imaging, and final confirmation is by pathology after surgical removal or biopsy in selected situations. Treatment depends on type, stage, hormone excess, and spread, and often involves surgery plus medicines and, in some cases, radiotherapy or systemic therapy. Key references give practical guidance on evaluation (biochemical tests such as plasma free metanephrines for pheochromocytoma; cortisol testing for ACC), risk stratification using histologic systems and Ki-67 index, and modern imaging and staging approaches. Oxford AcademicNational Cancer InstitutePMCese-hormones.org

A malignant adrenal gland tumor is a cancer that starts in one of the two adrenal glands. These glands sit above the kidneys and make important hormones, such as cortisol, aldosterone, and adrenaline-like chemicals (catecholamines). The two main cancers that begin in the adrenal area are adrenocortical carcinoma (ACC), which grows from the outer layer of the gland (cortex), and malignant pheochromocytoma or paraganglioma (PPGL), which grows from hormone-making nerve cells (medulla or nearby nerve tissue). A tumor is called “malignant” when it has spread to other parts of the body or shows aggressive behavior. Symptoms come from the mass itself or from too much hormone. The best chance for cure is complete surgical removal when possible. Some patients also need special medicines, targeted radiotherapy, or chemotherapy depending on tumor type and spread. Care is best in experienced centers. ESMOThe Lancet

Another names

Malignant adrenal gland tumor may also be called adrenal cancer, adrenocortical carcinoma (ACC), adrenal cortical carcinoma, malignant pheochromocytoma (when a catecholamine-producing adrenal medullary tumor proves malignant by metastasis), primary adrenal carcinoma, or simply adrenal malignancy. In clinical notes you may also see adrenal cortical neoplasm, malignant; for medullary disease, pheochromocytoma/paraganglioma (PPGL) with metastatic behavior. When cancer spreads to the adrenal from another organ (for example lung), it is termed adrenal metastasis rather than a primary adrenal cancer. National Cancer InstituteOxford Academic

Types

By tissue of origin

1. Adrenocortical carcinoma (ACC). Cancer of the adrenal cortex, often functional (makes excess cortisol and/or androgens) but can be nonfunctional. Histology uses scoring systems (Weiss, Helsinki) and proliferation measures such as mitotic rate and Ki-67 index to grade risk. PMCFrontiers

2. Malignant pheochromocytoma. A catecholamine-producing adrenal medullary tumor deemed malignant when it metastasizes to non-chromaffin sites (bone, liver, lung, nodes). It causes spells of headache, palpitations, sweating, and high blood pressure. Oxford Academic

3. Primary adrenal lymphoma or very rare primary sarcomas. Uncommon malignant tumors that begin in the adrenal. (Much rarer than ACC or PPGL.)

4. Adrenal metastasis (secondary). Not a primary adrenal cancer: cancers from lung, kidney, melanoma, colon, or breast commonly spread to the adrenal glands. These can mimic primary adrenal tumors on imaging.

By function

• Functional (hormone-secreting): Cushing’s syndrome from cortisol; virilization/feminization from androgens/estrogens; catecholamine excess from pheochromocytoma; rarely aldosterone excess from carcinoma.
• Nonfunctional: no hormone overproduction; often found incidentally on imaging (“incidentaloma”). Guidelines emphasize early determination of benign vs malignant features and functional status. ese-hormones.orgPubMed

Causes

Important context: For most people, the exact cause is unknown. A minority have inherited syndromes or tumor-driver mutations. What follows are recognized causes or strong risk conditions.

1. Li-Fraumeni syndrome (TP53). A hereditary mutation in the TP53 tumor suppressor raises ACC risk, especially in children and young adults. National Cancer Institute

2. Beckwith–Wiedemann spectrum and IGF2 dysregulation. Overgrowth syndromes with imprinting defects can lead to pediatric ACC.

3. Lynch syndrome (DNA mismatch repair). Germline MMR mutations increase multiple cancer risks and have been linked to ACC.

4. Multiple Endocrine Neoplasia type 2 (RET). Strongly associated with pheochromocytoma; a malignant course is less common but possible in some genotypes. Oxford Academic

5. Von Hippel–Lindau (VHL). Germline VHL changes predispose to pheochromocytoma; a fraction behave malignantly.

6. Neurofibromatosis type 1 (NF1). Raises risk for pheochromocytoma, occasionally with malignant behavior.

7. Succinate dehydrogenase (SDHB, SDHD, others) mutations. SDHB in particular is associated with a higher probability of metastatic PPGL. Oxford Academic

8. Familial paraganglioma syndromes. Overlaps with SDHx but includes additional PPGL susceptibility genes.

9. Pediatric genetic predisposition. Children in certain regions (e.g., southern Brazil with founder TP53 variants) have higher ACC rates. National Cancer Institute

10. Long-standing uncontrolled ACTH-independent cortisol excess from a neoplasm. This reflects tumor biology (functional ACC) rather than a cause; it signals malignant cortisol-secreting disease.

11. Prior radiation exposure (rare, uncertain). Weak, inconsistent data; not a proven driver but often listed among possible contributors.

12. Environmental or occupational exposures (uncertain). Suspected links (e.g., some industrial chemicals) lack definitive proof; research continues.

13. Male sex for PPGL in SDHB families. Some genotype–phenotype observations suggest differences in risk.

14. Older age for ACC. Sporadic ACC peaks in middle to older adulthood, suggesting age-related genomic instability.

15. Adrenal cortical hyperplasia from genetic syndromes. Overgrowth may serve as a step toward carcinoma in rare families.

16. Impaired DNA repair pathways. Beyond Lynch, other repair defects are being studied in ACC genomes. National Cancer Institute

17. High mitotic drive and Ki-67 elevation in neoplasm. Not a cause, but a hallmark of aggressive biology that predicts malignant behavior. PMC

18. RET-driven catecholamine tumorigenesis. In MEN2, mutant RET signaling drives medullary tumors that can progress. Oxford Academic

19. SDHB-driven pseudohypoxia signaling. Metabolic reprogramming in SDHB loss increases risk for metastatic PPGL. Oxford Academic

20. Unknown sporadic mutations. Most adult cases are sporadic; large sequencing efforts show diverse drivers, but no single environmental cause has been proven. National Cancer Institute

Symptoms

1. Persistent or severe high blood pressure. Especially paroxysmal spikes with headache, sweating, and palpitations suggest pheochromocytoma. Oxford Academic

2. Headache, pounding heart, and heavy sweating in spells. Classic “triad” of catecholamine excess (pheochromocytoma). Oxford Academic

3. Rapid heartbeat or irregular beats. From catecholamine surges and stress on the heart.

4. Unexplained weight loss, fatigue, loss of appetite. General cancer signs; also from excessive cortisol or catecholamines.

5. Features of Cushing’s syndrome. Round face, central weight gain, wide purple stretch marks, easy bruising, muscle weakness, mood change, diabetes and infections—often from cortisol-secreting ACC. National Cancer Institute

6. Signs of androgen excess in females. New facial/body hair, acne, deepening voice, menstrual changes due to androgen-secreting ACC.

7. Signs of estrogen excess in males. Breast enlargement, decreased libido, erectile dysfunction when ACC secretes estrogens.

8. Low potassium with muscle cramps or weakness. Rarely, ACC can produce aldosterone-like effects causing hypokalemia.

9. Abdominal, flank, or back pain/fullness. From a large adrenal mass pressing on nearby tissues.

10. Early satiety or bloating. Bulk effect of a big tumor.

11. New diabetes or worse glucose control. Cortisol and catecholamines raise blood sugar.

12. Anxiety or panic-like episodes. Driven by catecholamine surges.

13. Unexplained fevers or night sweats. Non-specific but may occur with aggressive disease.

14. Bone pain or cough with blood if spread. Symptoms depend on metastatic sites (bone, lung, liver).

15. Fragile skin and slow wound healing. Typical in hypercortisolism.

Diagnostic tests

Principle: Always exclude pheochromocytoma first with metanephrine testing before any procedure that could provoke a catecholamine crisis. Then evaluate cortisol, androgens/estrogens, and aldosterone/renin as indicated. Imaging should follow or accompany biochemical work-up. Final proof of malignancy rests on pathology for primary tumors; metastatic pheochromocytoma is defined by spread. Oxford Academic

A) Physical examination

1. Vital signs and blood pressure pattern. A clinician documents sustained hypertension or paroxysmal spikes with tachycardia; these suggest catecholamine excess and guide urgent precautions. Oxford Academic

2. General appearance for Cushingoid features. Rounded face, supraclavicular fat, proximal muscle weakness, and wide purple striae point to cortisol excess and prompt cortisol testing. National Cancer Institute

3. Skin and hair inspection. Hirsutism or acne in females suggests androgen excess; easy bruising and thin skin suggest cortisol excess.

4. Abdominal and back exam. Palpation for masses or tenderness and inspection for surgical scars or hernias; large tumors can sometimes be felt in thin individuals.

B) Manual (bedside) tests

5. Orthostatic vital sign test. Manual BP and pulse measured lying and standing can show postural changes common in catecholamine excess or cortisol-related volume changes.

6. Manual muscle strength testing. Proximal muscle weakness (hip/shoulder) supports hypercortisolism impact on muscles.

7. Waist circumference and body habitus assessment. Central adiposity with thin limbs favors cortisol excess and helps judge severity and metabolic risk.

8. Bedside neurologic screen. Reflexes, vibration, and sensation checks can reveal neuropathy or weakness from severe endocrine disturbance or metastasis.

C) Laboratory and pathological tests

9. Plasma free metanephrines (or urinary fractionated metanephrines). Best first test to rule in/out pheochromocytoma because metanephrines reflect continuous catecholamine metabolism; very sensitive. Positive results lead to imaging tailored for PPGL. Oxford AcademicNCBI

10. Overnight 1-mg dexamethasone suppression test. A late-evening pill should suppress morning cortisol in healthy physiology; failure suggests autonomous cortisol production from a functional adrenal tumor. PubMed

11. Late-night salivary cortisol. Elevated levels at bedtime suggest loss of normal rhythm and possible Cushing’s syndrome; easy, noninvasive screen. PubMed

12. Plasma ACTH. Low or undetectable ACTH with high cortisol points to an adrenal source (like ACC) rather than a pituitary source.

13. Androgen and estrogen panel (DHEA-S, testosterone, androstenedione, estradiol). Strong elevations—especially DHEA-S—support a cortical origin and can be striking in ACC. National Cancer Institute

14. Aldosterone-renin ratio (ARR). Consider if hypertension and low potassium suggest mineralocorticoid excess; carcinoma can rarely secrete aldosterone-like hormones. PubMed

15. Core pathology with histology and proliferation index (Ki-67) when tissue is available. Diagnostic scoring (Weiss, Helsinki; special systems for oncocytic or pediatric tumors) and Ki-67 labeling index (>5% typical for carcinomas) stratify malignancy risk and prognosis. (Biopsy is avoided if pheochromocytoma not excluded.) PMCPubMed

D) Electrodiagnostic / physiologic monitoring

16. 12-lead electrocardiogram (ECG). Detects tachyarrhythmias or ischemic changes caused by catecholamine surges; helps assess perioperative risk in PPGL and advanced ACC.

17. 24-hour ambulatory blood pressure monitoring. Characterizes paroxysmal or sustained hypertension, documents nocturnal patterns, and correlates symptoms with BP spikes in suspected PPGL.

E) Imaging tests

18. Adrenal-protocol CT scan. Non-contrast HU (Hounsfield units), contrast washout, and morphology help distinguish benign adenoma from carcinoma or metastasis; heterogeneity, irregular borders, necrosis, and high HU raise suspicion for malignancy. ese-hormones.orgPubMed

19. MRI with chemical-shift imaging. Useful when CT is indeterminate or contrast is contraindicated. Loss of signal on opposed-phase favors lipid-rich adenoma; carcinomas are usually lipid-poor and heterogeneous. ese-hormones.org

20. Functional imaging when indicated. For PPGL, ^123I-MIBG scintigraphy or PET (e.g., ^68Ga-DOTATATE, ^18F-FDG) helps localize disease and assess spread; ^18F-FDG PET/CT is also helpful for staging aggressive cortical carcinoma. Oxford AcademicNational Cancer Institute

Non-pharmacological treatments

(15 physiotherapy + mind-body, genetics & education). For each, you’ll see Description, Purpose, Mechanism, Benefits.)

Physiotherapy & exercise

1. Breathing training (diaphragmatic + paced breathing)
   Description: Short daily sessions to slow the breath and relax upper-chest muscles.
   Purpose: Reduce catecholamine-driven symptoms and anxiety spikes; support blood pressure control.
   Mechanism: Activates the parasympathetic system and baroreflexes; decreases sympathetic surges.
   Benefits: Fewer palpitations and headache episodes; better sleep and calm during evaluation and before surgery.

2. Gentle aerobic activity (walking, stationary cycling, water walking)
   Description: 20–30 minutes most days, adjusted for blood pressure and fatigue.
   Purpose: Maintain fitness, mood, and insulin sensitivity.
   Mechanism: Improves endothelial function and reduces inflammatory tone.
   Benefits: More stamina for surgery or chemotherapy; less fatigue in recovery.

3. Progressive resistance training (light weights or bands)
   Description: 2–3 sessions/week focusing on major muscle groups.
   Purpose: Prevent muscle loss from cortisol excess and inactivity.
   Mechanism: Stimulates muscle protein synthesis and counters steroid-related myopathy.
   Benefits: Stronger legs and core; easier daily activities.

4. Flexibility and joint mobility routines
   Description: 10–15 minutes of stretching after warm-up.
   Purpose: Reduce stiffness from inactivity or steroid effects.
   Mechanism: Lengthens muscle-tendon units; improves range of motion.
   Benefits: Better posture, less back pain.

5. Balance and fall-prevention training
   Description: Heel-to-toe walking, single-leg stands near support, tai chi-style drills.
   Purpose: Protect people with weakness or neuropathy from falls.
   Mechanism: Enhances proprioception and vestibular control.
   Benefits: Fewer injuries, safer home mobility.

6. Core stabilization and back care
   Description: Low-load abdominal and pelvic floor work; posture cues.
   Purpose: Support the spine if cough, weight changes, or surgery strain core muscles.
   Mechanism: Strengthens local stabilizers and improves intra-abdominal pressure control.
   Benefits: Less low-back ache, easier coughing and transfers after surgery.

7. Energy-conservation pacing
   Description: Break tasks, rest before tiredness, sit for chores.
   Purpose: Manage cancer-related fatigue.
   Mechanism: Prevents overexertion peaks that worsen next-day fatigue.
   Benefits: More steady energy through the week.

8. Neuromuscular electrical stimulation (when deconditioned)
   Description: Supervised sessions for weak muscles.
   Purpose: Preserve muscle mass if exercise is limited.
   Mechanism: Elicits contractions to maintain fibers.
   Benefits: Slows wasting during intensive therapy periods.

9. Gentle yoga or tai chi
   Description: Short, low-intensity sequences focused on breath and alignment.
   Purpose: Improve flexibility, balance, and calm.
   Mechanism: Combines movement with parasympathetic activation.
   Benefits: Less stress and better sleep quality.

10. Pelvic floor and posture retraining
    Description: Targeted cues after abdominal surgery.
    Purpose: Reduce pressure on incisions; improve continence and cough support.
    Mechanism: Activates deep core sling.
    Benefits: Smoother recovery and mobility.

11. Scar and rib-cage mobility after open adrenalectomy
    Description: Once healed, gentle soft-tissue work and breathing expansion.
    Purpose: Restore chest wall glide and diaphragmatic motion.
    Mechanism: Breaks adhesions; re-expands underused areas.
    Benefits: Easier deep breaths; less shoulder-side tightness.

12. Lymphedema-aware limb care (if nodes treated)
    Description: Skin care, compression as advised, and graded activity.
    Purpose: Reduce swelling risk after nodal surgery.
    Mechanism: Supports lymph return and skin barrier.
    Benefits: Fewer infections; better comfort.

13. Warm-up/cool-down routines
    Description: 5–10 minutes before/after exercise.
    Purpose: Smooth heart rate changes; prevent catecholamine surges.
    Mechanism: Gradual autonomic shift.
    Benefits: Safer sessions with fewer symptom spikes.

14. Sleep-hygiene coaching
    Description: Fixed schedule, dark cool room, limit late caffeine.
    Purpose: Improve restorative sleep, which is often poor with hormone excess.
    Mechanism: Resets circadian cues and reduces hyperarousal.
    Benefits: Better daytime energy, mood, and pain tolerance.

15. Return-to-work/activity planning
    Description: Stepwise plan with your team based on treatment.
    Purpose: Prevent setbacks and overuse.
    Mechanism: Graded exposure and pacing.
    Benefits: Higher quality of life with sustained activity.

Mind-body, genetics & educational therapies

16. Mindfulness-based stress reduction – lowers anxiety and sympathetic tone; helps blood pressure swings.

17. Cognitive behavioral therapy – reframes fear about spells or recurrence; improves coping and adherence.

18. Biofeedback (HRV or BP) – trains relaxation responses; can blunt catecholamine-triggered peaks.

19. Psycho-oncology counseling – addresses mood, relationships, and sleep; integrates caregiver support.

20. Guided imagery for procedures – improves pre-op calm and post-op pain coping.

21. Nutrition education – covers sodium/potassium goals (aldosterone states), glucose control (cortisol), and safe food handling during chemo.

22. Medication literacy – teaches mitotane timing with food, drug interactions, side-effect monitoring.

23. Genetic counseling – maps family risk, explains testing for PPGL genes and ACC syndromes, and sets screening plans.

24. Medical alert planning – wallet cards about PPGL and alpha-blockade; rescue steps for hypertensive crises.

25. Fatigue self-management program – bundles pacing, light exercise, sleep hygiene, and mood care.

(These supportive strategies complement—not replace—medical and surgical treatments.)

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Drug treatments

Important: medicines and doses must be individualized by specialists. The options differ between ACC and PPGL.

1. Mitotane (ACC)
   Class: Adrenolytic. Use: Backbone therapy in advanced ACC; may be used after surgery to lower recurrence risk in selected patients. Dose/Time: Titrated to blood levels; always with fatty meals to improve absorption. Purpose/Mechanism: Selectively damages adrenal cortex cells and alters steroid metabolism. Side effects: Nausea, diarrhea, fatigue, low cortisol requiring steroid replacement, neurologic effects; many drug interactions. It remains the only FDA-approved systemic therapy for ACC. PMC

2. EDP-M regimen (ACC) – Etoposide + Doxorubicin + Cisplatin with Mitotane
   Class: Cytotoxic combo + adrenolytic. Use: First-line for unresectable/metastatic ACC. Purpose/Mechanism: Multi-agent DNA damage and topoisomerase inhibition plus adrenolysis. Side effects: Low blood counts, infection risk, kidney injury (cisplatin), heart toxicity risk (doxorubicin), nausea, hair loss. The Lancet

3. Ketoconazole / Metyrapone / Osilodrostat (ACC cortisol control)
   Class: Steroidogenesis inhibitors. Use: Rapid control of high cortisol while cancer-directed therapy proceeds. Mechanism: Block cortisol synthesis at different steps. Side effects: Liver toxicity (ketoconazole), hypertension/hypokalemia (metyrapone/osilodrostat), drug interactions. (Guideline-supported for hypercortisolism management in ACC.) PubMed

4. Spironolactone / Eplerenone (ACC aldosterone control)
   Class: Mineralocorticoid receptor blockers. Use: Treat high blood pressure and low potassium due to aldosterone excess. Mechanism: Block aldosterone’s action on kidney salt handling. Side effects: High potassium, kidney effects; spironolactone can cause breast tenderness/gynecomastia. PubMed

5. Pembrolizumab (ACC selected cases)
   Class: PD-1 inhibitor immunotherapy. Use: Some heavily pretreated ACC patients experience responses. Mechanism: Reactivates anti-tumor T-cells. Side effects: Immune-related inflammation (thyroid, colon, liver, lungs). Response rates are modest overall (≈10–23% in small trials). PubMed+1Endocrine Abstracts

6. Streptozocin-based regimens (ACC, second-line)
   Class: Alkylating. Use: Alternative cytotoxic option when EDP-M is not suitable. Mechanism: DNA damage in tumor cells. Side effects: Nausea, kidney, and blood count effects. PubMed

7. Temozolomide (PPGL, especially SDHB-mutated)
   Class: Alkylating oral chemo. Use: Metastatic PPGL; activity may be higher in SDHB mutation. Mechanism: Methylates tumor DNA (MGMT-dependent). Side effects: Low counts, fatigue, nausea. National Cancer Institute

8. Cyclophosphamide + Vincristine + Dacarbazine (CVD) (PPGL)
   Class: Cytotoxic combo. Use: Longstanding regimen for malignant PPGL. Mechanism: Multi-agent cytotoxicity. Side effects: Low counts, neuropathy (vincristine), nausea. PMC

9. Sunitinib / Cabozantinib / Pazopanib (PPGL selected)
   Class: Multi-target tyrosine kinase inhibitors (anti-angiogenic). Use: For progressive metastatic PPGL in specialized care. Mechanism: Inhibit VEGF and other pathways that feed tumor blood vessels. Side effects: Hypertension, hand-foot syndrome, fatigue, diarrhea, thyroid issues. (Discussed in contemporary PPGL management reviews/guidance.) PMC

10. Iobenguane I-131 (Azedra®) (PPGL)
    Class: Targeted radiopharmaceutical (theranostic). Use: For MIBG-avid, unresectable or metastatic PPGL needing systemic therapy. Mechanism: Carries radioactive iodine into tumor cells via norepinephrine transporters, delivering internal radiation. Side effects: Low counts, nausea, fatigue; requires radiation safety measures. FDA-approved in 2018. U.S. Food and Drug AdministrationPMC

11. Phenoxybenzamine (alpha-blocker, PPGL)
    Class: Non-selective, non-competitive α-blocker. Use: First step before any surgery/procedure to prevent dangerous BP spikes. Mechanism: Blocks alpha receptors so catecholamines cannot cause vasoconstriction. Side effects: Low BP, stuffy nose, fatigue. Note: Beta-blockers are added only after alpha-blockade to control heart rate. National Cancer Institute

12. Doxazosin (alpha-1 blocker, PPGL)
    Class: Selective α1-blocker (alternative to phenoxybenzamine). Use/Mechanism/Effects: As above; shorter half-life, titratable. National Cancer Institute

13. Metyrosine (catecholamine synthesis inhibitor, PPGL)
    Class: Tyrosine hydroxylase inhibitor. Use: For difficult BP/heart-rate control or during crises; often added to alpha-blockade. Mechanism: Reduces catecholamine production at its first step. Side effects: Sedation, mood changes, crystalluria (hydration important). National Cancer Institute

14. Beta-blockers (e.g., propranolol, metoprolol) – after alpha-blockade
    Class: Beta-adrenergic blockers. Use: Control tachycardia in PPGL. Mechanism: Slow heart rate and reduce arrhythmias once vessels are protected. Side effects: Bradycardia, fatigue; avoid unopposed use before alpha-blockers. National Cancer Institute

15. Pain, nausea, and steroid replacement support
    Class: Multimodal supportive drugs (ondansetron, dexamethasone replacement when mitotane lowers cortisol, etc.). Use: Improve tolerance of cancer therapy and quality of life. Note: Steroid replacement must be carefully managed in patients on mitotane due to drug interactions. PubMed

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Dietary molecular supplements

Supplements can interact with cancer drugs (especially mitotane, TKIs, and chemo). Discuss every product with your specialists.

1. Vitamin D3 (1000–2000 IU/day, adjust to level): supports bone and immune health, often low with cortisol excess; check levels first.

2. Calcium (diet first; 500–600 mg/day in split doses if intake is low): protects bone in hypercortisolism or steroid replacement.

3. Omega-3 fatty acids (EPA/DHA 1–2 g/day with meals): may aid triglycerides, inflammation, and appetite; monitor bleeding risk with TKIs/anticoagulants.

4. Whey or plant protein (20–30 g once or twice daily): helps preserve lean mass during treatment; time around PT sessions.

5. Probiotics (clinician-approved strains/doses): may help antibiotic-related diarrhea; avoid if neutropenic.

6. Magnesium (200–400 mg/day): supports muscle and heart rhythm; monitor if on cisplatin (can cause low Mg).

7. Coenzyme Q10 (100–200 mg/day): sometimes used for fatigue; avoid near doxorubicin days without team approval.

8. Curcumin (up to 1–2 g/day standardized): anti-inflammatory properties; possible drug interactions (CYP effects); only with MD approval.

9. Green tea extract (EGCG ≤300 mg/day): antioxidant; may interact with proteasome and CYP pathways—use only if cleared.

10. Electrolyte-rich foods/supplements (potassium if low; sodium if directed): tailor to aldosterone or medication effects; follow lab guidance.

(These are supportive, not cancer cures.)

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Immune/regenerative/stem-cell–type” supportive drugs

(These are not cancer cures, but may support tolerability of treatment when your team prescribes them.)

1. Filgrastim (G-CSF) / Pegfilgrastim: boosts white cells after chemo to prevent infection; dosing per cycle schedule.

2. Erythropoiesis-stimulating agents (epoetin alfa, darbepoetin): may be used for chemo-related anemia in selected patients; careful risk–benefit.

3. Thrombopoietin-receptor agonists (eltrombopag, romiplostim): for low platelets in specific situations; specialist decision.

4. IV iron (ferric carboxymaltose) when iron-deficient: improves hemoglobin response; monitored for reactions.

5. Vaccinations (influenza, pneumococcal, COVID-19 as advised): reduce infection risk; time around immunosuppressive therapy.

6. Autologous stem-cell rescue (very rare in adrenal cancers): used only in unusual high-dose chemo contexts; not routine for ACC/PPGL.

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Surgeries and procedures

1. Open en-bloc adrenalectomy for suspected/confirmed ACC
   Procedure: Removal of adrenal tumor with surrounding fat ± adjacent organs if invaded, aiming for clear margins (R0).
   Why: Offers the best chance of cure; open approach lowers tumor spill risk. PMCThe Lancet

2. Laparoscopic adrenalectomy (selected small, non-invasive tumors, and many PPGLs)
   Procedure: Keyhole removal in experienced hands.
   Why: Less pain and faster recovery; not preferred for large or invasive ACC. PMC

3. Metastasectomy or cytoreductive surgery (ACC or PPGL)
   Procedure: Remove limited metastases (liver, lung) when feasible.
   Why: Can reduce hormone excess and improve symptom control; may extend disease control in selected cases. ESMO

4. Image-guided ablation (RFA/MWA) or stereotactic radiotherapy
   Procedure: Local treatments to burn or radiate small metastases.
   Why: Palliative control when surgery is not possible.

5. 131-I MIBG therapy setup (PPGL)
   Procedure: Diagnostic uptake test then therapeutic high-specific-activity MIBG given under radiation precautions.
   Why: Delivers targeted radiation to MIBG-avid tumors; FDA-approved for unresectable/metastatic PPGL. U.S. Food and Drug AdministrationPMC

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Practical preventions and safety tips

1. Genetic counseling/testing when PPGL or ACC is suspected, especially at young age or with family history.

2. Early alpha-blockade before PPGL surgery or invasive procedures to prevent dangerous crises. National Cancer Institute

3. Avoid unopposed beta-blockers in PPGL (can trigger hypertensive crisis). National Cancer Institute

4. Medication review to avoid drug interactions (mitotane has many; TKIs interact widely). PubMed

5. Carry a medical alert card (PPGL, steroid dependence on mitotane).

6. Sick-day rules for steroids if on replacement (stress-dose guidance).

7. Blood pressure monitoring at home during evaluation and treatment.

8. Heart health basics: no smoking, limit alcohol, regular gentle exercise.

9. Food safety during chemo or high-dose steroids (hand hygiene, avoid raw/undercooked foods).

10. Structured follow-up imaging and labs per specialist plan (recurrence surveillance). ESMO

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When to see doctors urgently or promptly

• Severe headache, chest pain, shortness of breath, or stroke-like symptoms, especially with very high blood pressure (possible PPGL crisis).

• Fever ≥38.0°C during chemo or on steroids (possible infection).

• Rapidly worsening weakness, confusion, or severe fatigue (possible cortisol swings or adrenal insufficiency).

• New severe abdominal pain, vomiting, or bleeding.

• New or worsening swelling in legs, sudden cough with chest pain (blood clots).

• Any new neurological symptoms (possible spread or metabolic issues).

• If pregnant or planning pregnancy and PPGL is suspected—see an expert team immediately. National Cancer Institute

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What to eat and what to avoid

1. Balanced meals with lean protein, whole grains, vegetables, and healthy fats to support recovery.

2. High-potassium foods (bananas, spinach, beans) if you have aldosterone-driven low potassium—confirm with your team.

3. Moderate sodium unless your doctor specifically advises higher salt (sometimes used during alpha-blockade). National Cancer Institute

4. Take mitotane with fatty foods to improve absorption (per specialist instruction). PubMed

5. Limit stimulants (excess caffeine, decongestants) that can worsen palpitations or BP spikes, especially in PPGL. National Cancer Institute

6. Stable carbohydrates (oats, legumes) and fiber to support blood sugar if cortisol is high.

7. Adequate calcium and vitamin D for bone health if cortisol is high or you use steroids.

8. Small, frequent meals if nausea; keep protein at each snack.

9. Avoid raw/undercooked foods when counts are low or during high-dose steroids.

10. Hydration (water, oral rehydration solutions) to protect kidneys during cisplatin-based therapy.

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Frequently asked questions (FAQs)

1) Is every adrenal mass cancer?
No. Most adrenal “incidentalomas” are benign. Features like large size, fast growth, hormone excess, and certain imaging traits raise concern and prompt specialist care. ESMO

2) What is the main cure for ACC?
Complete surgical removal with clear margins at high-volume centers offers the best chance for cure. Open en-bloc adrenalectomy is often recommended for suspected ACC. PMC

3) Why do PPGL patients get alpha-blockers before surgery?
To prevent dangerous blood-pressure surges during anesthesia or tumor handling. Beta-blockers are added only after alpha-blockade. National Cancer Institute

4) Is mitotane chemotherapy?
Mitotane is an adrenolytic drug that specifically injures adrenal cortex cells. It is the only FDA-approved systemic therapy for ACC. It often requires steroid replacement and close blood-level monitoring. PMC

5) Does immunotherapy work in ACC?
A minority of patients respond to PD-1 inhibitors like pembrolizumab; most do not. Studies show modest response rates overall. PubMed+1

6) What is 131-I MIBG and who gets it?
It is a targeted radiopharmaceutical used for MIBG-avid metastatic PPGL in patients who need systemic therapy. It was FDA-approved in 2018. U.S. Food and Drug Administration

7) Are there oral chemo options for PPGL?
Yes—temozolomide can be used, especially in SDHB-mutated disease, under specialist guidance. National Cancer Institute

8) Do all patients need genetic testing?
Many with PPGL do, because a large share have inherited mutations. Selected ACC patients may also need testing due to syndromic links. A genetics team will guide this. National Cancer Institute

9) If I feel well, can I skip alpha-blockade before PPGL surgery?
No. Even symptom-light PPGLs can surge during anesthesia or stress. Alpha-blockade is standard safety care. National Cancer Institute

10) Can diet cure adrenal cancer?
No diet can cure cancer. Nutrition supports strength, helps manage side effects, and protects bones and heart, but medical therapy and surgery are essential.

11) Is laparoscopic surgery safe for ACC?
Laparoscopy is common for small benign lesions and many PPGLs, but suspected large/invasive ACC is usually treated with open surgery to avoid tumor spillage. PMC

12) Will I always need chemotherapy for ACC?
Not always. Some patients have surgery alone; others need mitotane and/or chemo depending on stage, hormone excess, and margins.

13) How often will I need scans after treatment?
Follow-up schedules vary by tumor type and stage. Many patients get regular CT/MRI and hormone tests for years, especially in the first 2–3 years when recurrence risk is higher. ESMO

14) Are TKIs like sunitinib a first choice for PPGL?
They are options for progressive metastatic disease in expert centers, not first choice for all. Decisions depend on genetics, imaging avidity, and prior therapies. PMC

15) Where should I be treated?
Rare adrenal cancers do best in centers that see many such cases and can offer endocrine surgery, medical oncology, nuclear medicine, genetic counseling, and advanced imaging.

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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