A malignant adrenal medulla neoplasm is a cancer that starts in the inner part of the adrenal gland, called the medulla. This inner part makes “fight-or-flight” chemicals (catecholamines) such as adrenaline and noradrenaline. The cancer grows from special nerve-like cells called chromaffin cells. The tumor can make too much of these chemicals, which drives high blood pressure and many body symptoms. It is called malignant when it spreads to places where chromaffin cells do not normally live (for example, bones, liver, lungs, or lymph nodes). Malignancy is proven by spread, not just by how it looks under the microscope.
The adrenal medulla is the soft inner part of your adrenal gland. It makes stress chemicals called catecholamines (adrenaline, noradrenaline, dopamine). A malignant adrenal medulla neoplasm is a cancer that starts in these cells and can spread outside the adrenal gland to places like bone, liver, lung, or lymph nodes. The tumor often makes too many catecholamines. This causes sudden or constant high blood pressure, fast heartbeat, headache, sweating, pallor, tremor, and anxiety. Some people feel “adrenaline rush” spells. Malignancy is defined by metastasis (spread) or clear local invasion. The cancer can be slow or fast. Many cases are linked to inherited gene changes (for example, SDHB, RET/MEN2, VHL, NF1). Treatment usually combines alpha-blocker preparation, surgery, and—when spread is present—targeted radionuclide therapy, chemotherapy, tyrosine-kinase inhibitors, or clinical trials. Care is complex and needs a specialist team.
Other Names
This cancer is most commonly called malignant pheochromocytoma when it starts inside the adrenal gland. Closely related tumors that start outside the adrenal gland are called malignant paragangliomas (extra-adrenal). Other descriptive terms include adrenomedullary carcinoma, chromaffin cell carcinoma, or catecholamine-producing neuroendocrine carcinoma of the adrenal medulla. Doctors often use “PPGL” to cover both pheochromocytoma and paraganglioma. The key idea is the same: a neuroendocrine tumor that can release adrenaline-like hormones and is considered malignant when it spreads to distant, non-chromaffin sites. The name used depends on where the tumor begins and whether it has spread.
Types
1) By location
Adrenal (malignant pheochromocytoma): arises in the adrenal medulla on top of the kidney.
Extra-adrenal (malignant paraganglioma): arises from similar nerve tissues outside the adrenals (abdomen, chest, head/neck). Extra-adrenal tumors more often turn malignant than adrenal tumors.
2) By hormone pattern
Adrenergic type: mainly secretes adrenaline (epinephrine).
Noradrenergic type: mainly secretes noradrenaline (norepinephrine).
Dopaminergic type: mainly secretes dopamine (less common, sometimes linked with malignancy).
Non-secretory or low-secretory: little hormone release; symptoms may be mild or due to mass effect.
3) By behavior
Localized: confined to the adrenal gland.
Regionally invasive: grows into nearby tissues or lymph nodes.
Metastatic (malignant): spreads to bone, liver, lung, or distant lymph nodes (definitive sign of malignancy).
4) By genetics
Syndromic/hereditary: associated with germline variants (e.g., RET/MEN2, VHL, NF1, SDHx, MAX, TMEM127, FH, EPAS1/HIF2A, etc.).
Apparently sporadic: no known inherited variant; still, many “sporadic” tumors have tumor-only (somatic) mutations.
5) By pathology risk scoring
Systems such as PASS (Pheochromocytoma of the Adrenal gland Scaled Score) or GAPP (Grading system for Adrenal Pheochromocytoma and Paraganglioma) estimate risk of aggressive behavior using microscopic features and Ki-67 index. Note: only metastasis proves malignancy.
Causes
RET (MEN2A/MEN2B) germline variants
Changes in the RET gene cause multiple endocrine neoplasia type 2. These changes drive growth signals in chromaffin cells, increasing risk of adrenal medulla tumors, sometimes bilateral. Some can become malignant.VHL (von Hippel–Lindau) germline variants
VHL controls cell response to oxygen. When it fails, abnormal angiogenesis and growth occur, raising risk of pheochromocytoma or paraganglioma, occasionally malignant.NF1 (Neurofibromatosis type 1) germline variants
Loss of neurofibromin removes a tumor-suppressor brake on cell growth. This increases risk of adrenal medulla tumors, which may release catecholamines.SDHB germline variants
SDHB affects mitochondrial energy pathways. SDHB-related tumors have a higher chance of malignancy and distant spread, especially to bone and liver.SDHD germline variants
Alters energy metabolism and oxygen-sensing. Often linked to head/neck paragangliomas; some cases become malignant, especially when combined with other risk factors.SDHC germline variants
Less common than SDHB/SDHD but still part of the succinate dehydrogenase (SDH) family whose dysfunction promotes tumor growth.SDHA germline variants
Another SDH component; variants can drive PPGL formation and, in some cases, malignancy.SDHAF2 germline variants
A co-factor gene for SDH complex assembly. Variants disturb mitochondrial function and may promote tumor development.MAX germline variants
MAX regulates MYC signaling. Loss of normal control can increase tumor formation risk; some cases are bilateral or multiple.TMEM127 germline variants
Regulates mTOR signaling. Altered control of growth signaling can promote adrenal medulla tumor development.FH (fumarate hydratase) germline variants
Disrupts Krebs cycle, causing “pseudohypoxia” signaling and raising risk of PPGL; malignant behavior has been described.EPAS1/HIF2A germline/somatic variants
Stabilizes hypoxia-inducible factors, enhancing growth and blood vessel formation, sometimes linked with polycythemia and malignant PPGL.EGLN1/PHD2 variants
Impair oxygen-sensing enzymes, raising HIF activity and possibly driving tumor growth and aggressiveness.KIF1B variants
Involved in neuronal apoptosis; defective pathways can allow abnormal cell survival in chromaffin lineage.ATRX alterations (somatic)
Associated with chromatin remodeling and telomere maintenance; linked with more aggressive, metastatic PPGL in some series.MAML3 fusion events (somatic)
Lead to aberrant transcriptional signaling and have been observed in subsets of aggressive PPGL.HRAS mutations (somatic)
Activate MAPK signaling and can drive tumor formation; less often linked to metastasis but still relevant in some tumors.Chronic hypoxia states
Long-term low oxygen (e.g., cyanotic heart disease, high altitude living) may upregulate HIF pathways and promote PPGL development.Family history of PPGL
A strong predictor of hereditary risk; close relatives may carry the same germline variant.Prior radiation or environmental exposures (rare/uncertain)
Not a common cause, but any DNA-damaging exposure could, in theory, add risk; evidence is limited compared to genetic drivers.
Symptoms
High blood pressure (often severe or episodic)
Sudden hormone bursts tighten blood vessels and raise heart rate. Blood pressure may spike in attacks or stay high all the time.Headache
Rapid blood pressure surges and vessel changes in the head cause pounding, throbbing headaches, often with sweating and palpitations.Palpitations
Extra adrenaline makes the heart beat fast or irregular. People feel pounding, racing, or skipped beats in the chest or neck.Heavy sweating
Catecholamines stimulate sweat glands. People may wake soaked or suddenly sweat during attacks.Pallor or skin flushing
Sudden vessel changes can drain color (pallor) or occasionally cause facial flushing during hormone surges.Tremor and shaking
Excess adrenaline makes muscles twitch and shake, especially hands, and increases nervousness.Anxiety or panic-like episodes
Hormone spikes mimic panic attacks: rapid heart rate, chest tightness, short breath, fear, and a sense of doom.Weight loss
Fast metabolism and constant “fight-or-flight” state burn calories and reduce appetite, causing unplanned weight loss.Heat intolerance
The body runs “hot,” with sweating, palpitations, and restlessness. Warm environments feel uncomfortable.Nausea or vomiting
Sudden blood pressure changes and stress hormones can upset the stomach and trigger vomiting during attacks.Abdominal or flank pain
A large adrenal mass can press on nearby organs, causing dull or sharp pain in the upper abdomen or back.Chest pain or pressure
Strong heart stimulation and high blood pressure can cause chest discomfort; urgent care is needed to rule out heart injury.Shortness of breath
Rapid heart rate and high blood pressure strain the heart and lungs, making breathing feel hard during episodes.Blurred vision or visual spots
Blood pressure spikes can briefly reduce blood flow to the eyes or brain, causing blur, spots, or dizziness.Weakness or fainting (syncope)
Sudden pressure changes or rhythm problems may make a person light-headed or cause fainting, especially when standing.
Diagnostic Tests
A) Physical Examination (bedside observations)
Blood pressure in both arms, seated and standing
Doctors check for sustained high blood pressure and orthostatic changes (drop on standing). PPGL often causes stubborn hypertension and sometimes dramatic swings.Pulse and rhythm check
A fast, forceful, or irregular pulse during episodes suggests catecholamine surges. It guides urgency and next tests.Skin inspection (sweating, pallor, flushing)
Visible sweating, pale skin, or flushing during an attack supports a hormone-driven cause of hypertension.General survey (weight, anxiety, tremor)
Unplanned weight loss, visible tremor, and anxious appearance are simple clues pointing toward catecholamine excess.Abdominal and flank exam
A very large adrenal mass is rarely felt, but tenderness or fullness may appear and prompts imaging.
B) Manual / Bedside Tests (simple in-office maneuvers or monitoring)
Orthostatic vital signs (manual measurement)
Blood pressure and pulse are taken lying, sitting, and standing. PPGL may show labile pressure and exaggerated heart rate responses.Ambulatory blood pressure monitoring (24-hour)
A cuff records day-night blood pressures. PPGL can show paroxysmal spikes and loss of normal nighttime dipping.Home blood pressure diary
Frequent home readings capture attacks that clinic checks might miss, helping correlate symptoms with pressure surges.Trigger review test (history-based)
Clinician reviews triggers like certain drugs (decongestants), anesthesia, stress, or heavy exercise that can provoke attacks—this “test” is a structured, careful history.Medication challenge avoidance (safety practice)
Rather than provoke with drugs (which is unsafe), clinicians confirm that stopping risky medications before testing reduces false elevations and prevents crises.
C) Laboratory and Pathological Tests
Plasma free metanephrines
This is a first-line blood test. Metanephrines are breakdown products of catecholamines. High levels strongly suggest a secreting tumor.24-hour urine fractionated metanephrines and catecholamines
Collecting urine over a day measures hormones and metabolites during real life. Very useful if attacks are episodic.Plasma 3-methoxytyramine
A dopamine metabolite. High levels can point to dopaminergic or more aggressive tumors, including some malignant cases.Chromogranin A (CgA)
A general neuroendocrine marker. Elevated CgA supports the diagnosis but is nonspecific; acid-reducing drugs can falsely raise it.Electrolytes, glucose, kidney and liver tests
Check for high blood sugar from catecholamines, kidney issues from severe hypertension, or liver involvement if spread is suspected.Genetic testing panel (germline)
Because many PPGLs are inherited, testing for RET, VHL, NF1, SDHx, MAX, TMEM127, FH, EPAS1/HIF2A, and others guides care, family screening, and malignancy risk.Tumor pathology (after surgery or biopsy)
Under the microscope, pathologists confirm chromaffin-cell features and do immunohistochemistry: chromogranin and synaptophysin (positive), S100 for sustentacular cells, Ki-67 for growth rate. Loss of SDHB staining suggests SDH-mutated tumors with higher metastatic risk. PASS/GAPP scores estimate aggressiveness but do not prove malignancy.Biochemical preparation checks
Before surgery, repeating metanephrines after alpha-blockade helps confirm control of hormone release and reduces crisis risk under anesthesia.
D) Electrodiagnostic / Heart-Rhythm Tests
Electrocardiogram (ECG)
Looks for fast heart rhythm, strain patterns, or ischemia from severe hypertension. Abnormal ECG during symptoms supports catecholamine effects.Holter monitor (24–48-hour ECG)
Continuous rhythm recording detects runs of tachycardia, ectopy, or other arrhythmias linked to hormone surges. It helps explain palpitations and fainting.
(Note: Echocardiography is an imaging test, not electrodiagnostic, and is listed below.)
E) Imaging Tests
CT scan of the abdomen/pelvis with adrenal protocol
Fast and widely available. Shows tumor size, location, and relation to vessels. Can detect lymph nodes and some liver or lung spread.MRI of the abdomen (adrenal-focused)
Excellent tissue contrast without radiation. Typical PPGLs are very bright on certain MRI sequences. Helpful in younger patients and those with hereditary syndromes.^123I-MIBG scintigraphy (SPECT/CT)
Uses a norepinephrine-like tracer taken up by chromaffin cells. Positive uptake supports the diagnosis and can map disease throughout the body.^68Ga-DOTATATE PET/CT
Targets somatostatin receptors on many neuroendocrine tumors. Very sensitive for detecting lesions, especially in SDHx-related disease.^18F-FDG PET/CT
Shows high sugar uptake in aggressive tumors. Often lights up metastatic or fast-growing PPGLs, especially those with SDHB mutations.^18F-DOPA PET/CT
Amino-acid-based tracer taken up by catecholamine-producing cells; useful in selected cases to find small or extra-adrenal lesions.Chest CT
Checks for lung nodules and lymph nodes when distant spread is suspected.Bone scan or PET for bone metastases
Evaluates bone pain or elevated alkaline phosphatase; detects skeletal spread.Liver MRI or contrast CT
Detailed look at liver if lab or imaging suggests metastasis.Echocardiography (heart ultrasound)
Assesses heart muscle function and looks for stress-induced cardiomyopathy from prolonged catecholamine exposure.
Non-pharmacological treatments
Physiotherapy
Gentle aerobic conditioning (after alpha-blockade):
Description: Start with short walks or stationary cycling 10–20 minutes, 3–5 days/week once blood pressure is controlled with alpha-blockers. Increase time slowly. Avoid maximal exertion and heavy lifting until your team says it is safe. Use a home BP cuff before and after sessions.
Purpose: Improve heart fitness and reduce fatigue.
Mechanism: Aerobic work improves endothelial function, insulin sensitivity, and heart rate recovery; lowers resting sympathetic tone over time.
Benefits: Better stamina, mood, and sleep; safer daily activity tolerance.Breathing retraining (diaphragmatic):
Description: Sit or lie, one hand on chest, one on belly. Inhale through nose for 4–5 seconds, feel belly rise; exhale slowly through pursed lips for 6–8 seconds. Practice 5–10 minutes, 2–3 times/day.
Purpose: Cut the peak of catecholamine surges and anxiety.
Mechanism: Stimulates the vagus nerve, lowers heart rate and BP spikes.
Benefits: Fewer panic-like episodes; better control during spells.Isometric avoidance and graded resistance:
Description: Skip sustained heavy straining (e.g., max lifts, Valsalva) early on. Use light resistance bands with higher reps under guidance.
Purpose: Prevent BP surges.
Mechanism: Limits abrupt sympathetic spikes from heavy isometric load.
Benefits: Safer strength gains, joint support.Orthostatic tolerance training (post-op/post-blockade):
Description: If you get dizzy on standing, practice staged position changes: sit→stand with ankle pumps, small marches; compressive stockings if advised.
Purpose: Reduce postural dizziness.
Mechanism: Aids venous return; adapts baroreflex after alpha-blockade.
Benefits: Fewer near-faint episodes; confidence to walk.Flexibility and mobility work:
Description: Daily gentle stretching of hips, hamstrings, back, chest; 20–30 seconds/hold.
Purpose: Ease muscle tension from chronic catecholamine excess.
Mechanism: Reduces muscle spindle overactivity; improves circulation.
Benefits: Less headache/muscle tightness; better exercise form.Balance training:
Description: Heel-to-toe walks, single-leg stands near support, progressing as safe.
Purpose: Maintain stability if deconditioned or dizzy.
Mechanism: Trains proprioception and core.
Benefits: Fewer falls; safer mobility.Post-surgical early ambulation program:
Description: Short hallway walks day 1 if approved, then step counts.
Purpose: Reduce clots and lung issues; speed recovery.
Mechanism: Activates calf pump; improves ventilation.
Benefits: Shorter stay; faster return to normal.Pelvic and core stabilization:
Description: Low-load core exercises (e.g., dead bug, bridges) 3x/week.
Purpose: Support posture and reduce back pain from guarding.
Mechanism: Improves neuromuscular control.
Benefits: Comfort for daily activity and rehab.Thermal therapy for muscle tension:
Description: Warm packs to neck/shoulders 10–15 minutes, avoid extremes.
Purpose: Ease stress-related tension.
Mechanism: Vasodilation and muscle relaxation.
Benefits: Less headache and shoulder pain.Gentle yoga (restorative):
Description: Focus on supported poses and breath, not power yoga.
Purpose: Stress control and flexibility.
Mechanism: Parasympathetic activation.
Benefits: Calmer mood; better sleep.Aquatic therapy (if available):
Description: Walking in pool waist-deep for low-impact cardio.
Purpose: Joint-friendly conditioning.
Mechanism: Buoyancy reduces load; hydrostatic pressure aids venous return.
Benefits: Safe endurance with less joint strain.Headache self-management:
Description: Neck stretches, hydration plan, light-free rest room.
Purpose: Reduce triggers for catecholamine headaches.
Mechanism: Relieves muscle and autonomic triggers.
Benefits: Fewer severe headaches.Fatigue pacing:
Description: Plan day in blocks with rests; prioritize tasks.
Purpose: Manage low energy during treatment.
Mechanism: Prevents overexertion cortisol spikes.
Benefits: Consistent daily function.Bone-health exercises (if bone mets):
Description: Supervised, no high-impact; focus on hip/spine safety, posture.
Purpose: Reduce fracture risk.
Mechanism: Muscle support; improves balance.
Benefits: Safer mobility, less pain.Respiratory expansion drills post-op:
Description: Incentive spirometer or deep breaths hourly while awake.
Purpose: Prevent lung complications.
Mechanism: Re-expands alveoli.
Benefits: Lower pneumonia risk.
Mind-body, genetic, education & lifestyle
Trigger-avoidance education:
Description: Learn and avoid common triggers: decongestants, stimulants, energy drinks, tyramine-rich aged foods, intense isometric strain, extreme stress.
Purpose: Fewer spells.
Mechanism: Less sympathetic activation.
Benefits: Safer daily life.Stress-reduction skills (CBT, mindfulness):
Description: Short daily practice or brief CBT coaching.
Purpose: Cut anxiety loops that worsen spells.
Mechanism: Reframes threat cues; strengthens prefrontal control.
Benefits: Better calm, sleep.Sleep optimization plan:
Description: Fixed schedule, dark cool room, no late caffeine.
Purpose: Restore autonomic balance.
Mechanism: Improves baroreflex and cortisol rhythm.
Benefits: More energy, fewer surges.Hydration and salt guidance (during alpha-blockade):
Description: Follow team advice to increase fluids and salt before surgery.
Purpose: Prevent low BP after starting blockers.
Mechanism: Expands plasma volume.
Benefits: Safer standing and exercise.Family genetic counseling:
Description: Offer testing for RET, VHL, NF1, SDHx when indicated.
Purpose: Early detection and tailored follow-up.
Mechanism: Identifies at-risk relatives.
Benefits: Prevention and timely care.Medication literacy coaching:
Description: Teach sequence: alpha-blocker first, then beta-blocker.
Purpose: Avoid dangerous unopposed alpha activity.
Mechanism: Informed self-care.
Benefits: Fewer crises.Nutrition education for BP and sugars:
Description: Balanced meals, slow carbs, lean protein, healthy fats; avoid stimulants.
Purpose: Smooth glucose and BP.
Mechanism: Low glycemic load; stable insulin.
Benefits: Fewer swings.Return-to-work planning:
Description: Gradual hours; avoid high-stress roles early.
Purpose: Prevent relapse of spells.
Mechanism: Graded exposure.
Benefits: Sustainable recovery.Vaccination and infection prevention basics:
Description: Keep vaccines current; prompt care for infections.
Purpose: Reduce stressors that raise catecholamines.
Mechanism: Less systemic stress.
Benefits: Fewer setbacks.Palliative-care early integration (for advanced disease):
Description: Symptom control, pain, and quality-of-life support alongside treatment.
Purpose: Live better during therapy.
Mechanism: Multidisciplinary relief.
Benefits: Less distress, better goals-of-care planning.
Drug treatments
Phenoxybenzamine
Class: Non-selective, non-competitive α-blocker.
Use & Timing: First-line pre-operative BP control; start 10 mg twice daily and titrate every few days to effect (common total 40–100 mg/day). Begin 7–14 days pre-op.
Purpose/Mechanism: Blocks α-1 and α-2 receptors irreversibly, relaxing blood vessels and reducing catecholamine surges.
Side effects: Nasal stuffiness, fatigue, orthostatic dizziness, reflex tachycardia.Doxazosin (or Prazosin/Terazosin)
Class: Selective α-1 blocker.
Dose: Doxazosin 1 mg nightly → titrate to 8–16 mg/day; prazosin multiple daily doses.
Timing: Alternative to phenoxybenzamine; used pre-op and for long-term control.
Mechanism: Selective arteriolar/venous dilation with shorter action.
Side effects: Dizziness, headache; less reflex tachycardia than phenoxybenzamine.Beta-blocker (Propranolol or Metoprolol)
Class: β-adrenergic blocker.
Dose: Propranolol 10–40 mg three times daily; or metoprolol 25–100 mg twice daily. Start only after adequate α-blockade.
Purpose: Control tachycardia/arrhythmias.
Mechanism: Blocks β-1/β-2 (or β-1 selective) effects of catecholamines.
Side effects: Fatigue, bronchospasm (non-selective), bradycardia.Calcium-channel blocker (Amlodipine or Nifedipine)
Class: Dihydropyridine CCB.
Dose: Amlodipine 5–10 mg daily; nifedipine ER 30–60 mg daily.
Purpose: Add-on BP control if α-blocker alone is not enough.
Mechanism: Vascular smooth muscle relaxation.
Side effects: Ankle swelling, headache, flushing.Metyrosine
Class: Tyrosine hydroxylase inhibitor (catecholamine synthesis blocker).
Dose: 250 mg 3–4 times/day → titrate to 2–4 g/day as tolerated.
Purpose: Reduces catecholamine production in difficult cases or metastatic disease.
Mechanism: Blocks rate-limiting step in catecholamine synthesis.
Side effects: Sedation, depression, extrapyramidal symptoms, kidney stones (hydrate well).Magnesium sulfate (acute care)
Class: Mineral therapy with anti-adrenergic effects.
Dose: IV bolus then infusion per protocol in crises.
Purpose: Stabilizes BP and arrhythmias in intra-operative or crisis settings.
Mechanism: Inhibits catecholamine release; vasodilates.
Side effects: Flushing, hypotension, loss of reflexes at high levels.Sodium nitroprusside (crisis control)
Class: Potent IV vasodilator.
Dose: ICU titrated infusion.
Purpose: Rapid control of hypertensive crisis.
Mechanism: Nitric oxide donor → arterial/venous dilation.
Side effects: Hypotension, cyanide toxicity with prolonged high doses.Labetalol (selective use)
Class: Mixed α/β blocker.
Dose: IV bolus/infusion acutely; or oral for maintenance in selected cases after α-blockade is set.
Purpose: Manage peri-operative surges.
Mechanism: Blocks α-1 and β receptors (β>α potency).
Side effects: Hypotension, bradycardia; avoid as first drug because of unopposed alpha risk if used alone.ACE inhibitor / ARB (e.g., Lisinopril, Losartan)
Class: RAAS blocker.
Dose: Lisinopril 10–40 mg/day; losartan 25–100 mg/day.
Purpose: Background BP/heart protection when catecholamine control is adequate.
Mechanism: Blocks angiotensin pathway; reduces remodeling.
Side effects: Cough (ACEi), high potassium, kidney function changes.CVD Chemotherapy (Cyclophosphamide + Vincristine + Dacarbazine)
Class: Cytotoxic combination.
Dose: Given in cycles every 3–4 weeks in oncology centers (example: cyclophosphamide 750 mg/m² day 1, vincristine 1.4 mg/m² day 1, dacarbazine 600–750 mg/m² days 1–2).
Purpose: Shrink metastatic disease, relieve symptoms, lower catecholamines.
Mechanism: DNA damage and microtubule inhibition reduce tumor cells.
Side effects: Nausea, low blood counts, neuropathy, fatigue.Temozolomide
Class: Oral alkylating agent.
Dose: 150–200 mg/m² daily for 5 days every 28 days (common schedule).
Purpose: Option for metastatic disease, especially SDHB-mutated tumors.
Mechanism: DNA methylation causing tumor cell death.
Side effects: Fatigue, low counts, nausea.High-specific-activity ^131I-MIBG therapy
Class: Targeted radiopharmaceutical.
Dose: Administered in specialized centers with individualized GBq/mCi dosing and radiation safety protocols.
Purpose: Treat MIBG-avid metastatic disease; can reduce hormones and pain.
Mechanism: MIBG is taken up by norepinephrine transporters; delivers targeted beta radiation.
Side effects: Low blood counts, thyroid suppression (use iodine blockade), nausea.Peptide Receptor Radionuclide Therapy (PRRT, ^177Lu-DOTATATE)
Class: Radiolabeled somatostatin analog.
Dose: Commonly 7.4 GBq every 8 weeks × 4 cycles when SSTR-positive.
Purpose: Control growth and symptoms in receptor-positive tumors.
Mechanism: Binds somatostatin receptors on tumor → delivers radiation.
Side effects: Nausea, transient low counts, rare kidney effects (use renal protection).Sunitinib
Class: Tyrosine-kinase inhibitor (VEGFR, PDGFR).
Dose: 37.5–50 mg daily (continuous or 4-weeks-on/2-off per protocol).
Purpose: Slows tumor angiogenesis and growth in metastatic cases.
Mechanism: Blocks blood vessel growth signals.
Side effects: Fatigue, diarrhea, high blood pressure, hand-foot syndrome, thyroid dysfunction.Pembrolizumab (selected refractory cases/clinical trials)
Class: PD-1 immune checkpoint inhibitor.
Dose: 200 mg IV every 3 weeks (common).
Purpose: Attempt immune response against refractory disease; evidence evolving.
Mechanism: Lifts PD-1 brake on T-cells.
Side effects: Immune-related inflammation (thyroid, colon, liver, lung).
Note: Drug choices depend on tumor uptake (MIBG/SSTR), genes, prior therapy, and overall health. Your oncology and endocrine surgery teams individualize this.
Dietary molecular supplements
Omega-3 (EPA/DHA) – Dose: 1–2 g/day combined. Function/Mechanism: Anti-inflammatory, endothelial support; may help BP and heart health.
Vitamin D3 – Dose: 1000–2000 IU/day (or per level). Mechanism: Immune modulation, bone health; many adults are low.
Magnesium (glycinate/citrate) – Dose: 200–400 mg elemental/day. Mechanism: Smooth muscle relaxation; supports rhythm; caution with kidney disease.
Coenzyme Q10 – Dose: 100–200 mg/day. Mechanism: Mitochondrial support; may reduce statin-like fatigue.
Curcumin (high-absorption) – Dose: 500–1000 mg/day with food. Mechanism: Anti-inflammatory signaling modulation; avoid before surgery if bleeding risk.
Psyllium fiber – Dose: 5–10 g/day with water. Mechanism: Smoother glucose and lipid control.
Probiotics – Dose: per label (e.g., 10–20 billion CFU/day). Mechanism: Gut-immune axis support; may help bowel regularity.
Selenium – Dose: 50–100 mcg/day (mind total intake). Mechanism: Antioxidant enzymes; thyroid support.
B-complex (with B12/folate) – Dose: per label. Mechanism: Nerve and energy metabolism; stress support.
Electrolyte solution (low-sugar) – Dose: as needed for hydration during alpha-blockade. Mechanism: Volume expansion for orthostatic symptoms.
Avoid stimulant supplements (yohimbine, high-caffeine “fat burners,” synephrine, ephedra).
Immunity booster / regenerative / stem-cell” drugs
There are no approved stem-cell drugs that cure malignant adrenal medulla cancer. Some supportive or immune-modulating treatments are used in special settings:
Influenza and pneumococcal vaccination – reduces infection stress during treatment. Dose: per national schedule. Mechanism: Prepares immune system; prevents complications.
G-CSF (filgrastim/pegfilgrastim) – used with chemotherapy to raise white cells. Dose: per cycle protocol. Mechanism: Stimulates bone-marrow neutrophils; lowers infection risk.
Checkpoint inhibitors (e.g., pembrolizumab) – see above; not a booster, but modulates T-cells against tumor when appropriate.
Bone-protective agents (zoledronic acid or denosumab) – for bone metastases. Dose: monthly or q12 weeks. Mechanism: Strengthen bone, reduce skeletal events.
Clinical-trial cellular therapies – investigational only; discuss at major centers. Mechanism: Experimental immune redirection.
Nutritional optimization plus exercise – not a drug, but the best real-world “immune support.” Mechanism: Improves host resilience and treatment tolerance.
Surgeries
Adrenalectomy (laparoscopic):
Procedure: Small incisions, camera, instruments remove the tumor and gland when size and anatomy allow.
Why: Curative for localized disease; fewer complications and faster recovery.Adrenalectomy (open):
Procedure: Larger incision for big, invasive, or anatomically complex tumors.
Why: Better control, safe margins, vascular management.Lymphadenectomy or local re-resection:
Procedure: Remove involved nodes or residual disease.
Why: Staging, debulking, symptom relief.Metastasectomy (liver/lung/bone):
Procedure: Select removal or ablation (RFA, microwave, cryo) of deposits.
Why: Hormone control, pain relief, slower progression.Spine stabilization/vertebroplasty:
Procedure: Cement or hardware for painful or unstable bone mets.
Why: Pain control, prevent fracture/neurologic injury.
Before surgery, careful alpha-blockade and volume expansion are essential to prevent dangerous BP spikes.
Preventions
You cannot fully prevent a genetic tumor, but genetic counseling finds risks early.
Regular screening in high-risk families (labs + imaging schedule).
Avoid sympathomimetic drugs (certain decongestants, stimulants).
Limit high-caffeine/energy drinks.
Avoid heavy isometric straining until cleared.
Control stress with daily brief practices.
Healthy weight and activity to support BP and glucose.
Medication adherence (alpha first, then beta if needed).
Keep a spell diary to learn and avoid triggers.
Early evaluation in pregnancy if hypertension or classic symptoms appear.
When to see doctors
Call emergency services NOW for severe headache with very high BP, chest pain, sudden shortness of breath, severe palpitations, confusion, fainting, or signs of stroke.
Contact your team soon for new or worsening spells, sustained BP above targets, new bone pain, weight loss, or medication side effects.
Routine follow-up for lab checks (metanephrines), imaging, and drug adjustments; family members may need genetic counseling and screening.
What to eat and what to avoid
What to eat
Balanced meals with lean protein, vegetables, whole grains, healthy fats.
Adequate fluids and salt during alpha-blockade if your team recommends it.
Slow-carb choices (oats, legumes) to steady glucose.
Potassium-rich foods (unless restricted): bananas, leafy greens.
Calcium and vitamin D sources for bone health.
What to avoid
- Energy drinks and high-caffeine shots (trigger surges).
- Decongestants with pseudoephedrine/phenylephrine unless approved.
- Aged/fermented high-tyramine foods (aged cheeses, cured meats) that may worsen surges in sensitive people.
- Alcohol excess (can trigger BP changes).
- Large heavy meals close to bedtime (worsen BP and reflux).
Frequently asked questions
Is every adrenal medulla tumor cancer?
No. Many are benign, but malignancy means it has spread or invades nearby tissues.What is the main clue?
High blood pressure with spells of headache, sweating, and palpitations.What test is best to start?
Plasma free metanephrines or 24-hour urine fractionated metanephrines.Why block alpha before beta?
Beta-blockers first can leave unopposed alpha effect and cause a dangerous BP spike.Can surgery cure it?
Yes, if localized. If it has spread, surgery can still debulk and improve symptoms.What if it comes back?
Options include MIBG therapy, PRRT, chemotherapy, TKIs, or trials.Do I need genetic testing?
Often yes, because many cases are hereditary and this guides follow-up.Will exercise make it worse?
With BP controlled and a tailored plan, gentle exercise helps. Avoid heavy straining.Are supplements enough?
No. Supplements are adjuncts only and should be cleared with your team.How are metastases found?
With CT/MRI plus functional PET/SPECT scans (MIBG, DOTATATE, FDG, FDOPA).How often do I need scans after surgery?
Your team sets a schedule, commonly every 6–12 months at first, then individualized.Can pregnancy be safe?
It can be high risk. Early diagnosis and team care are critical.What about blood sugar?
Catecholamines raise glucose; control often improves after treatment.Does diet cure the tumor?
No. Diet helps symptoms and heart health, but not the tumor itself.Where should I be treated?
At a center with endocrine oncology, experienced surgeons, and access to radionuclide therapy.
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The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: September 09, 2025.
