Severe Hypertension in a 14-Year-Old Boy

Inas H. Thomas, MD

Disclosures

May 31, 2017

Definitive treatment requires surgery, usually performed laparoscopically, although this depends on the size of the tumor.[6] Patients with pheochromocytomas should be medically prepared 1-2 weeks before surgery, to reduce the potential for severe catecholamine fluctuations. Alpha-blockade is initiated with phenoxybenzamine or prazosin. This reduces the blood pressure and increases the blood volume in the vascular beds. Starting with low doses and carefully increasing doses to minimize symptomatic postural hypotension is important.[1,6,11]

Once alpha-blockade is achieved, beta-blockade can be instituted to reduce reflex tachycardia. A beta-blocker should not be used alone because it can cause hypertension, owing to unrestricted catecholamine effects at the alpha receptors that result in vasoconstriction. Before surgery, intravenous fluids or salt-loading is recommended to increase the vascular volume in order to reduce postoperative hypotension.[1,6,11] Routine hydrocortisone administration is not needed, because these patients are not adrenally insufficient.

In most cases, surgery is definitive, with removal of the benign tumor. Further treatment is directed at managing postoperative hypotension.[6] If both adrenal glands are removed, the patient will then have adrenal insufficiency that requires replacement hydrocortisone treatment.[6]

If the tumor is localized and can be completely removed, the prognosis is favorable, with a 5-year survival rate greater than 95%.[1] In patients with malignant tumors, chemotherapy may be required, which could decrease the size of the tumor.[6] The 5-year survival rate is quoted to be 34%-60%, which is decreased in those with poor response to chemotherapy and those with lung and liver metastases.[1,6]

In this case, the 14-year-old boy was found to have a localized pheochromocytoma. He responded well to preoperative medical management, and the tumor was completely removed. Owing to his family history, he and his mother were tested and found to have VHL syndrome.

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