Pheochromocytoma in Children, Adolescents, and Teenagers

Do Children and Teenagers Get Pheochromocytomas?

Pheochromocytoma (and paraganglioma) are relatively rare tumors in children, adolescents, and teenagers, but yes, they DO occur. The incidence of pheochromocytoma occuring in children and the pediatric population is around 0.2–0.3 cases per million. Most of these pheo tumors are clinically symptomatic (they DO have symptoms) which are due to the hypersecretion of catecholamines (adrenaline) and their metabolites. Pheochromocytomas in children typically cause hypertension, palpitations, sweating, and headaches.

Bilateral (both left and right) pheochromocytomas seen on a CT scan (arrows) in a young patient with inherited pheochromocytomas due to multiple endocrine neoplasia type 2A (MEN2A). The patient underwent successful Partial Cortical-Sparing Adrenalectomy via Mini Back Scope Adrenalectomy (MBSA). Children with High Blood Pressure Should be Checked for Pheochromocytoma. About 1% of pediatric cases and children with arterial hypertension suffer from a pheochromocytoma or paraganglioma as the cause of the high blood pressure. The majority of the patients with tumors arising during childhood are carriers of germ line mutations of the susceptibility genes (in brackets) which predispose to von Hippel–Lindau syndrome (VHL), Multiple Endocrine Neoplasia type 2 (RET), Neurofibromatosis type 1 (NF1), Paraganglioma Syndromes type 1–5 (SDHD, SDHAF2, SDHC, SDHB, SDHA), MYC-associated factor X (MAX), hypoxia-inducible factor 2A (HIF2A), fumarate hydratase (FH), and transmembrane protein 127 (TMEM127). Learn more about the molecular genetics of pheochromocytoma and paraganglioma on our pheo-gentics page.

Are Pheochromocytomas in Children Malignant or Cancer?

Most of pheochromocytoma and paraganglioma tumors are benign. However, the risk of malignancy is related to the patient having an underlying genetic mutation and these occur mainly in patients carrying mutations of the SDH(x) gene. Most of these are children. So even though the vast majority of pheochromocytomas in children are beining, they can be malignant and genetic testing is necessary. This is why you need to be seen by an expert in adrenal surgery.

What is the Best Surgery and Operation for Pheochromocytomas in Children?

Surgery, either open or by endoscopic (scope adrenalectomy) approach, is the treatment of choice in pheochromocytoma in children and teenagers. Just as with adults the Mini Posterior Retroperitoneal Scope Adrenalectomy (Mini-PRSA, also called the Mini-Back-Scope Adrenalectomy) is the preferred technique in most of pediatric adrenal surgery cases. (Dr. Carling editorial; If you have a child with pheochromocytoma or paraganglioma, please find the most experienced surgeon in the world to perform the operation to avoid unnecessary complications). Please don't let your local surgeon do these operations on your children.

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The diagnosis of pheochromocytoma in children hinges on the treating physician thinking about the diagnosis in the first place. Making the diagnosis is usually straightforward by performing the following tests measuring adrenaline type hormones in the blood and urine:

• Measurement of plasma free metanephrines and normetanephrines (blood test). This is often an accurate test with sensitivity of 97-100% and a specificity of 85-89%. Plasma free metanephrines levels above 3-4 times normal almost always indicates the presence of pheochromocytoma.
• 24-hour collection of urine fractionated metanephrines and catecholamines. This is often an accurate test and has a high sensitivity and specificity and can be used for confirmatory testing or in lieu of plasma testing.

It is important to know that many medications may affect (increase) adrenaline type hormones so it is important that you tell us and your doctor what medications (if any) you were on while being tested. The list of the medication that may interfere with measuring adrenaline type hormones is on the "Lab Testing" for pheochromocytoma page.

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