Severe Hypertension in a 14-Year-Old Boy

Inas H. Thomas, MD


May 31, 2017


This patient with severe hypertension has either a pheochromocytoma or paraganglioma (PGL), which is a neuroendocrine tumor that produces catecholamines.[1] Pheochromocytomas arise from the adrenal medulla and were known by the "10% rule," which indicated that 10% of these tumors are bilateral, familial, malignant, and/or extra-adrenal.[2]

Extra-adrenal tumors, also known as "PGLs," arise from sympathetic and parasympathetic PGL and can be found anywhere from the skull to the abdomen.[1,2] PGLs have a higher rate of malignancy than pheochromocytomas, but both can result in morbidity from the space-occupying lesion.[2]

As genetic testing has improved, one third to one half of patients with pheochromocytomas are now believed to have a hereditary form of pheochromocytoma.[2,3] The incidence is estimated to be about 1 case per 300,000 individuals; however, in children who are hypertensive, pheochromocytomas have been reported to account for approximately 1.7% of cases.[1,2,4]

Patients usually present with headaches, hypertension, and diaphoresis. The hypertension may fluctuate, depending on intermittent release of the catecholamines due to inciting stresses, such as surgery, foods, and medications.[2]

Tremor and anxiety are common, as well as deterioration in schoolwork in children.[1,5] This can mimic attention-deficit/hyperactivity disorder; thus, screening for hypertension in children evaluated for attention-deficit/hyperactivity disorder is important.[5] Starting treatment with a stimulant medication in a child with an undiagnosed pheochromocytoma could potentially lead to an adrenergic crisis.[5]

Patients may also present with various seemingly nonrelated symptoms, such as abdominal pain, diarrhea, weight loss, hearing loss, fever, polyuria, and hyperglycemia. Some of these symptoms may be related to where the tumor is located, if it is extra-adrenal.[1,6] An example is shown in Figure 2.

Figure 2.

The diagnosis is determined by measuring fractionated plasma or urine metanephrines and normetanephrines. Plasma-free metanephrines are preferred over urine studies; results greater than four times the reference range are consistent with the diagnosis of a catecholamine-producing tumor.

Before testing, patients should discontinue medications, such as acetaminophen, decongestants, and tricyclic antidepressants, because they may affect the assay. In addition, to minimize false-positive results from stress and exercise, the blood sample should not be collected until about 30 minutes after the needle is present in the vein, while the patient is supine. If the results are inconclusive in a patient who appears to have a high probability of having a pheochromocytoma, blood samples should be obtained soon after a hypertensive episode.[1,6]


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