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Pheochromocytoma

A rare neuroendocrine tumor secreting adrenaline and noradrenaline from the adrenal medulla or sympathetic ganglia.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Pheochromocytoma?

Pheochromocytoma is a rare neuroendocrine tumor that secretes catecholamines (adrenaline, noradrenaline, sometimes dopamine) from the adrenal medulla — or from sympathetic ganglia in extra-adrenal locations as paragangliomas. Incidence is 2-8 per million per year and it is found in approximately 0.1-0.6% of hypertensive patients.

About 10% are malignant, and 30-40% are linked to autosomal dominant hereditary syndromes (MEN2, Von Hippel-Lindau, neurofibromatosis type 1, SDH mutations). The classic 'rule of 10s' applies: 10% extra-adrenal, 10% bilateral, 10% malignant, 10% hereditary.

Diagnosis is made by measuring 24-hour urinary or plasma free metanephrines (the most sensitive method) along with imaging (CT/MRI). Imaging is not initiated before biochemical confirmation.

Symptoms

Hypertensive crises (sudden, severe paroxysmal blood pressure spikes)
Palpitations (tachycardia and arrhythmia)
Excessive sweating attacks
Headaches (frequent and severe)
Pallor or flushing
Tremor and feelings of anxiety
Weight loss and hyperglycemia

Risk Factors

MEN2A or MEN2B syndrome (RET mutation)
Von Hippel-Lindau disease
SDH subunit gene mutations (SDHB, SDHD, etc.)
Neurofibromatosis type 1
Family history of pheochromocytoma or paraganglioma
Previously identified adrenal incidentaloma

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Hypertension with crisis-like blood pressure spikes
  • The triad of palpitations, sweating, and headache occurring together
  • Detection of an adrenal mass (incidentaloma evaluation)
  • Family history of MEN2 or pheochromocytoma — for screening

Treatment Methods

01
Preoperative alpha blocker (phenoxybenzamine or doxazosin): mandatory for at least 7-14 days for blood pressure control
02
Beta blocker added after alpha blockade: for tachycardia control (never administered without prior alpha blockade!)
03
Laparoscopic adrenalectomy: curative surgery once catecholamines are stabilized
04
I-131 MIBG therapy or chemotherapy in malignant/metastatic pheochromocytoma
05
Genetic screening of family members in hereditary forms
06
Postoperative plasma metanephrine follow-up: for recurrence monitoring

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.