Endocrine surgery · KIMS Secunderabad
Pheochromocytoma — The Adrenal Tumour That Causes Hypertensive Crises
Pheochromocytoma (pheo) is a tumour of the chromaffin cells of the adrenal medulla — the inner portion of the adrenal gland — that produces and secretes catecholamines: adrenaline (epinephrine), noradrenaline (norepinephrine), and dopamine. The excess catecholamine secretion — which may be continuous or episodic — drives the clinical syndrome of phaeochromocytoma: paroxysmal or sustained hypertension, often severe; palpitations; profuse sweating; severe headaches; and pallor or anxiety attacks. It is the only hypertension-causing condition that can kill a patient intra-operatively if not diagnosed and prepared for correctly.
Phaeochromocytoma is rare — occurring in approximately 2 to 8 per million population per year — but it is clinically critical to identify because: it causes severe, treatment-resistant hypertension with significant cardiovascular morbidity; it is entirely curable by surgical removal; and operating on it without appropriate pre-operative preparation causes life-threatening intra-operative hypertensive crises. The 'rule of 10' historically described pheochromocytoma: 10% bilateral, 10% extra-adrenal (paraganglioma), 10% malignant, 10% familial — in practice, genetic associations are found in 30 to 40% of cases with modern testing.
Symptoms — the classic triad and its variants
The classic paroxysmal triad. Episodic headache (often severe, throbbing) + sweating + palpitations occurring simultaneously. This triad, particularly in a hypertensive patient, has high specificity for phaeochromocytoma. Episodes may last minutes to hours and occur spontaneously or triggered by: stress, exercise, bending, bladder distension, medications (beta-blockers — which can precipitate crisis by blocking beta-mediated vasodilation, leaving unopposed alpha-vasoconstriction; contrast dye; certain anaesthetic agents; tricyclic antidepressants).
Sustained hypertension — in 50% of patients, hypertension is persistent rather than episodic. Phaeochromocytoma should be considered in any patient with resistant hypertension, particularly if young or with associated symptoms.
Pallor — from profound noradrenaline-driven vasoconstriction. Hypertension with pallor (rather than facial flushing — which suggests carcinoid) is characteristic of phaeochromocytoma.
Hyperglycaemia — catecholamines stimulate gluconeogenesis and glycogenolysis, causing diabetes or worsening of existing diabetes.
Weight loss — hypermetabolic state from catecholamine excess.
Incidental detection — increasingly, phaeochromocytoma is found as an incidental adrenal mass on CT. Every adrenal mass must be screened for phaeochromocytoma before surgery.
Diagnosis
Plasma metanephrines (normetanephrine and metanephrine)
The recommended first-line biochemical test — sensitivity above 97% for phaeochromocytoma. Metanephrines are the metabolic by-products of catecholamine breakdown produced continuously within the tumour cells (unlike catecholamines themselves, which are released episodically). A resting, supine blood sample with specific laboratory handling. Elevated plasma metanephrines confirm the biochemical diagnosis.
24-hour urine catecholamines and metanephrines
Alternative first-line test — measures total catecholamine and metanephrine output over 24 hours. Slightly lower sensitivity than plasma metanephrines but still highly sensitive. Preferred where plasma metanephrine testing is not available.
CT of the abdomen — anatomical localisation
Once biochemical diagnosis is confirmed, CT (or MRI) localises the tumour. Adrenal phaeochromocytomas are typically large (4 to 6cm), heterogeneous (often with central necrosis), hypervascular, and show avid enhancement with contrast. MRI with T2-weighted imaging is particularly useful — phaeochromocytomas are characteristically 'bright' (high signal) on T2 imaging.
MIBG scintigraphy or DOTATATE PET-CT
Functional imaging to detect extra-adrenal tumours (paragangliomas) or metastatic disease. MIBG (metaiodobenzylguanidine) is taken up by chromaffin tissue. DOTATATE PET-CT is increasingly preferred for its superior sensitivity.
Genetic testing
Recommended for all phaeochromocytoma patients — 30 to 40% have a germline mutation in SDHB, SDHD, VHL, RET (MEN2), NF1, or TMEM127. SDHB mutation carriers have a 50% risk of malignant phaeochromocytoma. Genetic testing determines surveillance requirements for both the patient and family members.
Pre-operative preparation — the critical safety step
Every patient with a confirmed phaeochromocytoma must undergo alpha-adrenergic blockade before surgery — without exception. This is the most important surgical safety step in all of adrenal surgery.
Alpha-blockade (phenoxybenzamine or doxazosin)
Started 10 to 14 days before surgery. Blocks alpha-1 and alpha-2 adrenergic receptors, preventing the vasoconstriction from catecholamine release during tumour manipulation. The patient may experience postural hypotension and nasal stuffiness as desired side effects — confirming adequate blockade.
Beta-blockade (propranolol or atenolol)
Added after adequate alpha-blockade is established (never before alpha-blockade, to avoid unopposed alpha-mediated vasoconstriction causing hypertensive crisis). Controls tachycardia and arrhythmias.
High-sodium diet and fluid loading
Prevents the postural hypotension that occurs as the alpha-blockade expands the vascular bed. Initiated alongside alpha-blockade and continued through to the day of surgery.
Surgical treatment — robotic adrenalectomy at KIMS
Laparoscopic or robotic adrenalectomy is the standard surgical approach for phaeochromocytoma. At KIMS, Dr. Likhiteswer Pallagani performs robotic adrenalectomy using the Da Vinci Xi or X — 3 to 4 small incisions, 1 to 2 night hospital stay, return to normal activity within 1 to 2 weeks. The key surgical principle: minimal manipulation of the tumour before the adrenal vein is ligated — early vein ligation reduces the catecholamine surge during dissection. The anaesthesiology team at KIMS uses specific protocols (phentolamine or sodium nitroprusside available immediately) to manage intra-operative blood pressure swings.
After phaeochromocytoma removal, blood pressure may drop precipitously — from the sudden loss of catecholamine excess. IV fluids and vasopressors are prepared preoperatively. Blood pressure is monitored continuously in the post-operative period.
Book a Pheochromocytoma Assessment at KIMS — Alpha-Blockade Protocol and Robotic Surgery
Frequently Asked Questions — Pheochromocytoma
A phaeochromocytoma paroxysm — a sudden surge of catecholamine release from the tumour — typically lasts minutes to an hour and consists of: severe throbbing headache, intense sweating (drenching, not localised), palpitations (rapid, forceful heartbeat), pallor, and extreme anxiety or a sense of impending doom. Blood pressure during the attack may reach 250/150 mmHg or higher. Episodes can be precipitated by physical exertion, bending, emotional stress, bladder distension, or — importantly — certain medications (beta-blockers, contrast dye, tricyclic antidepressants, and some anaesthetic agents). If you experience this cluster of symptoms together, particularly with resistant hypertension, seek medical evaluation at KIMS.
Yes — phaeochromocytoma is associated with catecholamine cardiomyopathy (a form of stress cardiomyopathy from chronic excess catecholamine exposure), severe hypertension-related cardiac damage, and acute hypertensive crises that can cause myocardial infarction, aortic dissection, or intracerebral haemorrhage. Phaeochromocytoma is one of the conditions that must be excluded in any patient presenting with a hypertensive emergency, particularly if accompanied by sweating, palpitations, and headache. The cardiovascular complications of undiagnosed phaeochromocytoma are the primary reason why early diagnosis and surgical cure are so important.
Most pheochromocytomas (approximately 85 to 90%) are benign — they do not metastasise. Malignant phaeochromocytoma is defined by the presence of metastases (to lymph nodes, bone, liver, or lung) rather than by histological features of the primary tumour — all phaeochromocytomas look similar under the microscope. SDHB mutation carriers have the highest risk of malignant phaeochromocytoma (approximately 50%). Surveillance after surgery includes annual biochemical testing (plasma metanephrines) and periodic imaging to detect recurrence or metastasis — particularly important for SDHB carriers.
Beta-blockers must never be started before adequate alpha-blockade is established in phaeochromocytoma. Beta-1 blockade reduces heart rate and cardiac output. Beta-2 blockade blocks the vasodilatory beta-2 receptors on peripheral blood vessels. When both are blocked with unopposed alpha-1 activation from catecholamine excess, systemic vasoconstriction is intensified dramatically — causing severe, potentially fatal hypertensive crisis. The correct sequence is always alpha-blockade first (phenoxybenzamine or doxazosin for 10 to 14 days) — then beta-blockade added to control the resulting reflex tachycardia.
KIMS Secunderabad — Dr. Likhiteswer Pallagani (Vattikuti Foundation uro-oncology fellowship, 400+ robotic cases), plasma metanephrine testing, complete pre-operative alpha-blockade protocol (phenoxybenzamine or doxazosin), robotic adrenalectomy with specific intra-operative catecholamine surge management protocol, genetic testing for SDHB/VHL/RET/NF1 mutations, post-operative surveillance programme. Da Vinci Xi AND X. NABH and NABL accredited. Call 040-4488-5000.