Phaeochromocytoma surgery, by a specialist adrenal surgeon.

Prices vary by unit, by which surgeon and anaesthetist take the case, and by tumour size, side and whether cortical-sparing or open resection is required. London centres in our panel include King’s College Hospital Private, Guy’s and St Thomas’ Private Endocrine Surgery, Hammersmith Imperial Private Endocrine and HCA The Wellington.

1. 01

   Before

   You send us the biochemistry and imaging

   A short, confidential form. Plasma or urinary metanephrines, adrenal-protocol CT or MRI, and any functional imaging or genetic testing already done.

2. 02

   Before

   We come back with an MDT plan

   Within one working day: a named surgeon, anaesthetist and endocrinologist, an indicative price, and whether extra imaging or genetics is needed first.

3. 03

   Before

   Alpha-blockade for two to three weeks

   Phenoxybenzamine is titrated upward, with a high-salt diet and volume expansion. Beta-blockade is added only once alpha-blockade is established.

4. 04

   Admission and surgery

   Admission the day before surgery

   Overnight IV fluids at around one litre per hour. Potassium and magnesium are corrected. Arterial and central venous lines placed in theatre.

5. 05

   Admission and surgery

   Laparoscopic or open adrenalectomy

   A specialist endocrine anaesthetist manages intraoperative catecholamine surges with phenylephrine, sodium nitroprusside and short-acting agents.

6. 06

   Admission and surgery

   HDU for 24 to 48 hours

   Close monitoring for rebound hypotension once the tumour is out. Vasopressors are weaned as adrenoreceptors reset.

7. 07

   After

   Histology, genetics and lifetime follow-up

   Histology at 7 to 14 days. Clinical genetics if not already arranged. Annual metanephrines and blood pressure for life given recurrence risk.

• Classic triad with hypertension

  Episodic headaches, palpitations and sweating with paroxysmal or sustained hypertension is the textbook presentation.

• Pallor, anxiety and weight loss

  Pale skin during spells, unexplained anxiety, weight loss and glucose intolerance often accompany a secreting tumour.

• Orthostatic hypotension paradox

  A hypertensive patient who drops sharply on standing is a classic phaeo clue caused by volume contraction.

• Incidentaloma over 4cm or lipid-poor

  An adrenal lesion picked up on scanning with a non-adenoma density needs biochemical screening before any biopsy.

• Family history or known germline mutation

  SDHx, VHL, RET, NF1, TMEM127, MAX or FH carriers need screening even when asymptomatic.

• Extra-adrenal or head-and-neck paraganglioma

  Tumours arising from paraganglia in the neck, chest or abdomen need dedicated imaging and often FDG-PET.

• Refractory or crisis hypertension

  Blood pressure that will not settle on three or more agents, or a hypertensive crisis under anaesthesia, warrants screening.

• Red flag: suspected metastatic disease

  Bone pain, weight loss or lymphadenopathy with raised metanephrines needs 68Ga-DOTATATE PET-CT and specialist oncology input.

• Alpha-blockade must come first

  Phenoxybenzamine 10 to 40 mg three times daily is titrated over two to three weeks with a high-salt diet. Beta-blockade added only after this is established.

• Never beta-blockade alone

  Unopposed alpha stimulation triggers a hypertensive crisis. Propranolol or atenolol is added three to five days after alpha-blockade if tachycardia persists.

• Preoperative targets

  Supine blood pressure under 120 over 80, an acceptable postural drop, and supine heart rate under 80. A calcium channel blocker may be added.

• Intraoperative catecholamine surges

  Tumour manipulation releases catecholamines. Sodium nitroprusside, phenylephrine and short-acting beta-blockers are drawn up before incision.

• Rebound hypotension after resection

  Once the tumour is out, catecholamine drive stops while adrenoreceptors remain down-regulated. HDU observation for 24 to 48 hours is standard.

• Bilateral disease and Addison’s risk

  Cortical-sparing adrenalectomy is preferred when both glands are involved to avoid lifelong steroid replacement.

• Malignant phaeochromocytoma

  Around 10 to 15 per cent metastasise. Treatment involves 131I-MIBG, CVD chemotherapy, sunitinib or 177Lu-DOTATATE PRRT with five-year survival of 50 to 90 per cent.

• Lifetime surveillance

  Annual metanephrines, blood pressure and calcium. Germline carriers need mutation-specific screening for life given recurrence risk.

• Red flags after discharge

  Severe headache, chest pain, palpitations, sweating or a hypertensive spike above 180 over 110 needs the on-call team or A&E the same day.