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Adrenal Pheochromocytoma and Paraganglioma Treatment

Surgical and systemic management of 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 Onkoloji department. Book Appointment →

What is Adrenal Pheochromocytoma and Paraganglioma Treatment?

Pheochromocytoma arises in the adrenal medulla and paraganglioma from extra-adrenal sympathetic or parasympathetic ganglia. They secrete catecholamines and produce paroxysmal hypertension, headache, palpitations, and sweating, with a substantial proportion driven by germline mutations in SDHx, VHL, RET, NF1, and other susceptibility genes.

Diagnosis combines plasma free metanephrines, twenty-four-hour urinary fractionated metanephrines, and cross-sectional imaging followed by functional imaging with metaiodobenzylguanidine or DOTATATE positron emission tomography. All patients should be referred for genetic counseling because hereditary syndromes affect surveillance and family screening.

Treatment requires careful preoperative blockade with alpha-adrenergic antagonists, salt loading, and beta blockade only after adequate alpha blockade is achieved. Minimally invasive adrenalectomy is the gold standard for benign disease. Metastatic disease may respond to high-specific-activity iodine-131 metaiodobenzylguanidine, sunitinib, or other systemic therapies.

Symptoms

Episodic severe headache with sweating and palpitations
Sustained or paroxysmal hypertension
Pallor and anxiety during episodes
Postural hypotension after volume depletion
Adrenal incidentaloma on imaging

Risk Factors

Germline SDHx, VHL, RET, or NF1 mutations
Family history of pheochromocytoma or paraganglioma
Multiple endocrine neoplasia type 2 syndromes
Von Hippel-Lindau or neurofibromatosis 1
Carney triad with paraganglioma and gastrointestinal stromal tumor

When to See a Doctor?

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

  • When paroxysmal hypertension and headaches occur
  • When an adrenal mass is detected on imaging
  • When family history suggests hereditary syndrome
  • When postural symptoms or arrhythmia occur during anesthesia
  • When metastatic disease is suspected on follow-up

Treatment Methods

01
Plasma and urinary metanephrine measurement
02
Functional imaging with MIBG or DOTATATE
03
Preoperative alpha-adrenergic blockade with phenoxybenzamine
04
Beta blockade only after adequate alpha blockade
05
Minimally invasive adrenalectomy or paraganglioma resection
06
Lifelong biochemical surveillance
07
Genetic testing and family counseling

Which Department to Visit?

You can visit our Onkoloji 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.