Pheochromocytoma is a rare tumor of the inner portion of the adrenal gland tissue that results in the release of too much epinephrine and norepinephrine that are hormones that control heart rate, metabolism, and blood pressure. Very few pheochromocytomas are cancerous. They occur from early to mid-adulthood.

This condition occurs most often within the 3rd and 5th decades of life. It may be seen in conjunction with medullary thyroid cancer and parathyroid adenomas.

Hypertension is present in 95% of patients with pheochromocytoma. Frequent symptoms include: excessive perspiration, postural hypotension, increased heart rate, flushing, cold and clammy skin, severe headache, angina, palpitations, nausea, vomiting, spasms of severe pain in the epigastric area, visual disturbances, dyspnea, paresthesias, constipation, and a sense of impending doom. Emotional trauma, postural changes, and compression of the abdomen may provoke attacks.

The metabolic products of epinephrine and norepinephrine in the urine are tested. Normally, very small amounts are excreted in the urine. In this condition, levels increase sporadically. Computed tomography (CT) scan and magnetic resonance imaging (MRI) are used to localize tumors.

Surgical removal of a benign tumor is the treatment of choice. Malignant metastatic tumors (cancer), are treated with chemotherapy.

Complete removal of benign tumors cures the hypertension in 75% of patients. In the remaining 25%, the recurrence of hypertension is controlled with antihypertensive drugs. Inoperable metastatic tumors will continue to grow. Radiotherapy is not effective. In addition, little success results from chemotherapy. The five-year survival rate is 50%.

• Consider service connection on a presumptive basis as a chronic condition (38 CFR §3.307; §3.309 (a)). 

• Consider service connection on a presumptive basis as a condition associated with ionizing radiation exposure (38 CFR §3.307; §3.309 (d); §3.311 (b)).

• Evaluate as malignant or benign neoplasm, as appropriate.