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Pheochromocytoma in Children

A rare adrenal tumor in childhood that secretes catecholamines and causes hypertension.

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 Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Pheochromocytoma in Children?

Pheochromocytoma is a tumor of the adrenal medullary chromaffin cells; in childhood it is rare but more frequently bilateral, multifocal and extra-adrenal (paraganglioma) than in adults. About 60–70% of cases have a hereditary basis.

It is most often associated with VHL syndrome, MEN 2A/B, neurofibromatosis type 1 (NF1) and SDHA/B/C/D mutations. Hypertension is most often persistent rather than paroxysmal in children, and accompanies symptoms such as headache, sweating and palpitations.

Diagnosis is made with metanephrines (plasma free or 24-hour urine) and imaging (MRI, MIBG scintigraphy, 68Ga-DOTATATE PET). Treatment is surgical resection, with absolutely required preoperative alpha-blockade (10–14 days) and adequate hydration.

Symptoms

Persistent hypertension
Headache (severe)
Sweating
Palpitations and tachycardia
Pallor
Weight loss
Visual disturbances

Risk Factors

VHL syndrome
MEN 2A/2B
Neurofibromatosis type 1
SDHA/B/C/D mutations
Family history
Bilateral adrenal tumor

When to See a Doctor?

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

  • Persistent hypertension in children
  • Headache + sweating + palpitations triad
  • Rapid growth and weight loss
  • Family history of MEN/VHL
  • Adrenal mass (incidental)
  • Hypertensive crisis findings

Treatment Methods

01
Preoperative alpha-blockade (phenoxybenzamine)
02
Beta-blocker (after alpha)
03
Adequate hydration
04
Laparoscopic adrenalectomy
05
Genetic testing and family screening
06
Long-term endocrine follow-up

Which Department to Visit?

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

Learn About Çocuk Sağlığı ve Hastalıkları Department

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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.