Pheochromocytoma is a rare neuroendocrine tumor in the medulla of the adrenal glands. The tumor originates from the chromaffin cells or chromaffin tissue outside of the adrenal glands that have failed to involute after birth. These cells or tissue secrete high amounts of catecholamines which mostly consist of norepinephrine and some epinephrine. It has been estimated that about 85 percent of these tumors are in the adrenal glands with 98% located within the abdomen. Pheochromocytomas outside the adrenal gland are known as extra-adrenal pheochromocytomas (paragangliomas). Common locations for paragangliomas are the bladder wall, mediastinum, heart, carotid, and more.
Approximately 30% of patients have this condition due to hereditary syndromes. Pheochromocytoma is malignant in 10 percent of cases and is classically associated with multiple endocrine neoplasia type 2 (MEN 2), neurofibromatosis type 1 (NF1), and von Hippel-Lindau (VHL) syndrome. Diagnosis involves plasma metanephrine testing, 24-hour urinary collection, abdominal computed tomography (CT) scan, magnetic resonance imaging (MRI), scintigraphy, screening for genetic mutations, and more. Diagnosis is important as consequences can be fatal if the diagnosis of pheochromocytoma is overlooked. The treatment of this condition involves surgical resection of the tumor.
Preoperative medical stabilization is required to control blood pressure. It is estimated that about 10 percent of pheochromocytomas are malignant. Pheochromocytomas have been reported to occur in all races and ages. The incidence peaks among those in their 30s to 50s. Ten percent of pheochromocytomas occur in children. The prognosis is excellent with a 5-year survival rate of 95 percent in non-malignant cases while it drops to less than 50 percent in malignant cases.
Symptom #1: Severe Hypertension
Hypertension or high blood pressure occurs when the blood pressure in the arteries are consistently elevated. Chronic hypertension is a risk factor for many conditions such as stroke, heart failure, coronary heart disease, vision loss, chronic kidney disease, atrial fibrillation, dementia, and more.
In pheochromocytoma, the elevated blood pressure can be persistent or sporadic. It is also the commonest sign found in 95 percent of patients with pheochromocytoma.