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Pheochromocytoma in children

William F Young, Jr, MD, MSc
Section Editors
Alberto S Pappo, MD
Mitchell Geffner, MD
Deputy Editor
Alison G Hoppin, MD


Pheochromocytomas and paragangliomas are rare neoplasms in children. Tumors that arise from the adrenal medulla are termed pheochromocytomas, and those with extra-adrenal origins are called paragangliomas. Among hypertensive children, the incidence of surgically confirmed disease has ranged from 0.8 to 1.7 percent [1-3]. Because of the uncommon nature of the disease, few published reports document more than 15 cases even during prolonged observation periods [4-6]. Given the limited experience with pheochromocytomas in children and the similarity in clinical presentation, diagnosis, and treatment among children and adults, the following discussion includes some data derived from the adult literature.


The signs and symptoms of pheochromocytomas and paragangliomas are caused by tumoral hypersecretion of norepinephrine, epinephrine, and dopamine, although increased central sympathetic activity also may contribute [7]. The classic triad of symptoms in these disorders consists of episodic headache, sweating, and tachycardia, usually accompanied by hypertension. However, only 50 percent of adult patients have one or more of the "three classic symptoms" [8]. Symptoms caused by the mass effect of the tumor, such as abdominal pain and distension or back pain, were found in 30 percent of children in one study; 64 percent of the patients had hypertension [6]. Most children have sustained hypertension rather than paroxysmal [1,9]. Malignant hypertension can occur with its associated complications (eg, increased intracranial pressure, encephalopathy) [1,10,11].

Other signs and symptoms that occur less frequently include pallor, orthostatic hypotension (which may reflect a low plasma volume), constipation, psychiatric disorders, blurred vision, weight loss, polyuria, polydipsia, increased erythrocyte sedimentation rate, hyperglycemia, and a dilated cardiomyopathy [1,7,12].

The clinical presentation is often different when pheochromocytoma is associated with the multiple endocrine neoplasia type 2 (MEN2) syndrome. Symptoms are present in only approximately one-half of patients, and only one-third have hypertension [13]. Whether this difference is due to ascertainment bias from screening for pheochromocytomas in families with MEN2 or a real difference in the clinical expression of the disease is not known. A similar finding has been observed with pheochromocytoma associated with von Hippel-Lindau (VHL) disease, as 35 percent of patients have no symptoms, a normal blood pressure, and normal catecholamine tests [14]. (See "Clinical features, diagnosis, and management of von Hippel-Lindau disease" and "Clinical manifestations and diagnosis of multiple endocrine neoplasia type 2".)

Compared to adults, children with pheochromocytomas have a higher incidence of bilateral adrenal tumors, extraadrenal tumors, and multiple tumors [1,4,15]. In different series, extraadrenal tumors have been described in 30 to 60 percent of children (contrasted to 10 to 15 percent of adults) [6,9,16,17], and multiple tumors have been described in up to 40 percent (compared to 5 to 10 percent in adults) [18]. Multicentric disease is more common with familial disorders such as MEN2 and mutations in succinate dehydrogenase (SDH; succinate:ubiquinone oxidoreductase) and subunit genes (SDHB, SDHC, SDHD, SDHAF2, SDHA) [19,20]. Catecholamine-secreting paragangliomas are co-located with chromaffin tissues (eg, along the para-aortic sympathetic chain, within the organs of Zuckerkandl at the origin of the inferior mesenteric artery, wall of the urinary bladder, and the sympathetic chain in the neck or mediastinum) [21,22].


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Literature review current through: Sep 2016. | This topic last updated: Aug 8, 2016.
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