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Pheochromocytoma and Paraganglioma

Catecholamine-secreting neuroendocrine tumors.

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 Internal Medicine department. Book Appointment →

What is Pheochromocytoma and Paraganglioma?

Pheochromocytomas arise from the adrenal medulla, while paragangliomas originate from extra-adrenal sympathetic or parasympathetic ganglion cells. Both are catecholamine-secreting neuroendocrine tumors. A genetic predisposition is identified in 30-40% of patients (SDH, RET, VHL, NF1 mutations).

The classic triad consists of paroxysmal headache, sweating, and palpitations. Hypertension may be sustained or paroxysmal. Plasma free metanephrines and 24-hour urine metanephrines have high sensitivity for diagnosis.

Adequate preoperative alpha-adrenergic blockade (phenoxybenzamine or doxazosin) is mandatory to prevent intraoperative catecholamine crisis. The malignancy rate is 10% in pheochromocytoma and 15-35% in paraganglioma.

Symptoms

Paroxysmal headache (severe)
Excessive sweating
Palpitations and tachycardia
Hypertension (paroxysmal or sustained)
Anxiety and panic-attack-like episodes
Pallor (due to vasoconstriction)

Risk Factors

Genetic syndromes (MEN 2, VHL, NF1, SDH mutations)
Family history of pheochromocytoma
Hypertension at a young age
Adrenal incidentaloma
Paradoxical hypertension (triggered by anesthesia or drugs)

When to See a Doctor?

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

  • If you have paroxysmal episodes of headache, sweating, and palpitations
  • If resistant or paroxysmal hypertension is found
  • If an adrenal mass is detected
  • If there is a family history of pheochromocytoma or a related genetic syndrome

Treatment Methods

01
Surgery (laparoscopic adrenalectomy — curative treatment)
02
Preoperative alpha-blockade (phenoxybenzamine or doxazosin, 10-14 days)
03
Subsequent addition of beta-blockade (only after alpha-blockade)
04
In malignant cases: chemotherapy (CVD regimen), MIBG therapy
05
Genetic counseling and family screening

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.