Paraganglioma

Neuroendocrine tumors originating from sympathetic or parasympathetic nervous system paraganglia outside the adrenal gland.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 10, 2026

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

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 Paraganglioma?

Paragangliomas are neuroendocrine tumors originating from paraganglia outside the adrenal gland. Head and neck paragangliomas (carotid body, jugulotympanic, vagal) are generally parasympathetic-derived and non-secretory. Paragangliomas in the abdomen and pelvis are sympathetic-derived and can secrete catecholamines.

30-40% of paragangliomas are hereditary and may be associated with SDHx (SDHA, SDHB, SDHC, SDHD, SDHAF2) gene mutations, VHL, NF1, and RET genes. Cases carrying SDHB mutations show more aggressive and metastatic course.

Plasma and urinary metanephrines, chromogranin A, and functional imaging methods such as 68Ga-DOTATATE PET/CT are used in diagnosis.

Symptoms

Episodic headache, palpitations, sweating (classic triad)

Paroxysmal hypertension

Anxiety and panic-attack-like symptoms

Mass and cranial nerve findings in head-neck paragangliomas

Abdominal pain (in abdominal tumors)

Syncope and orthostatic hypotension

Weight loss and fatigue

Risk Factors

Family history of paraganglioma or pheochromocytoma

SDHx gene mutations

VHL, NF1, and MEN 2 syndromes

Carney-Stratakis syndrome

Diagnosis at early age (<40)

Multiple or bilateral tumors

Long-term high altitude exposure

When to See a Doctor?

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

Resistant or episodic hypertension
Pulsatile mass in head-neck region
Hypertension at young age
Classic triad (headache, palpitations, sweating)
Screening in those with family history

Treatment Methods

Preoperative α and β-blockade in catecholamine-secreting tumor

Surgical resection (first choice)

131I-MIBG or 177Lu-DOTATATE in metastatic cases

Chemotherapy (CVD regimen) in selected cases

Targeted therapy (TKI such as sunitinib)

Genetic testing 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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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Paragangliomas are rare neuroendocrine tumors arising from paraganglia outside the adrenal glands, distinct from but related to pheochromocytomas. Recognition of hereditary syndromes, comprehensive biochemical evaluation and meticulous perioperative management are essential for optimal outcomes in these uncommon catecholamine-secreting tumors.

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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.