Page 51 Complete Your CE Test Online - Click Here ● ● Total plasma catecholamines: Levels are 10 to 50 times above normal. ● ● Clonidine suppression test: In normal patients, results show decreased plasma catecholamine levels. However, in those persons with pheochromocytoma, levels remain unchanged. ● ● CT scans or MRIs: Imaging tests are used to identify tumor location. Analysis of a 24-hour urine specimen is used to confirm a diagnosis of pheochromocytoma[5] . “Increased urinary excretion of total free catecholamines and their metabolites, VMA and metanephrine, as measured by analysis of 24-hour urine specimen, confirms pheochromocytoma[5] .” Nursing consideration: To makes sure that urine catecholamine measurements are reliable, nurses must teach patients to avoid foods high in vanillin for 2 days before urine collection of VMA. Examples of such foods include coffee, nuts, chocolate, and bananas[5] . Treatment The treatment of choice for pheochromocytoma is surgical resection of the tumor, which usually cures the hypertension[31] . It is important that specific preoperative measures be taken beginning 1 to 2 weeks prior to surgery to control blood pressure and prevent intraoperative hypertensive crisis[5,31] . Measures to achieve preoperative medial stabilization include[31] : ● ● Administration of an alpha-adrenergic blocker or metyrosine. ● ● Volume expansion with isotonic sodium chloride solution. ● ● Facilitation of liberal salt intake. ● ● Administration of a beta blocker only after sufficient alpha blockade to “avoid precipitating a hypertensive crisis from unopposed alpha stimulation[31] .” ● ● Administration of the last doses of oral alpha and beta blockers on the morning of surgery. Postoperatively, the following measures are taken[5] : ● ● Administration of intravenous fluids. ● ● Administration of plasma volume expanders. ● ● Administration of vasopressors. Persistent hypertension is possible in the period immediately following surgery as are acute episodes of headaches, palpitations, anxiety, and diaphoresis. Management of hypertensive crisis or acute episodes includes administration of push or drip intravenous phentolamine or nitroprusside to control and normalize blood pressure[5] . For persons who are not able to withstand surgery, alpha-adrenergic blockers and beta-adrenergic blockers are administered to help control the effects of catecholamine and to prevent acute episodes[5] . Treatment alert! Rarely, pheochromocytoma is malignant. If this is the case, surgery to excise the tumor is followed by radiation and/or chemotherapy as needed[5,31] . Additional postoperative nursing considerations include[5,31] : ● ● Monitor blood pressure meticulously. If hypertensive crisis occurs, blood pressure and heart rate should be monitored every 2 to 5 minutes until the patient stabilizes. ● ● Monitor glucose levels. ● ● Provide a quiet, calm environment since anxiety, noise, and excitement can trigger a hypertensive episode. ● ● Keep the room cool since postoperative adrenal gland secretions cause profuse diaphoresis. Change clothing and bed linens frequently. Treatment alert! Blood pressure may also drop drastically in the postoperative period, especially during the first 24 to 48 hours after surgery. Monitor for signs of severe hypotension[5] . If the occurrence of pheochromocytoma is due to a suspected autosomal dominant transmission, family members should also be tested for this problem[5] . Multiple endocrine neoplasia Multiple endocrine neoplasia (MEN) is an inherited disorder in which 2 or more of the endocrine glands develop hyperplasia, an adenoma, or a malignancy. These pathologies can occur at the same time or consecutively[5] . There are 2 types of MEN that are well recorded[5] : ● ● MEN I, also called Werner’s syndrome, occurs because of a defect in a gene that carries the code for the protein menin[32] . This defect leads to hyperplasia and tumors of the pituitary and parathyroid glands, islet cells of the pancreas, and, rarely, the thyroid and adrenal glands[5] . ● ● MEN II, also called Sipple’s syndrome, is a rare familial malignancy caused by genetic mutation[33] . It usually involves medullary cancer of the thyroid and hyperplasia and tumor growth of the adrenal medulla and parathyroid glands[5,33] . MEN I is the more common form[5] . Incidence and etiology MEN is usually due to autosomal dominant inheritance. It affects twice as many females as males and can occur at any time from adolescence through old age. However, it is rare in children[5] . Signs and symptoms Clinical manifestations of MEN depend on the glands involved. The most common signs and symptoms of MEN I are those of hyperparathyroidism, including hypercalcemia, followed by ulcer development because of increased production of gastrin from non- beta islet cell tumors of the pancreas (Zollinger-Ellison syndrome). Hypoglycemia may occur as a result of pancreatic beta cell tumors that lead to increased production of insulin[5] . Here is a list of possible signs and symptoms related to MEN I[5,32] : ● ● Abdominal pain. ● ● Amenorrhea. ● ● Anxiety. ● ● Black, tarry stools. ● ● Confusion. ● ● Decreased appetite. ● ● Decreased libido. ● ● Epigastric pain relieved by eating or taking antacids. ● ● Fatigue. ● ● Feeling bloated after eating. ● ● Headache. ● ● Loss of facial hair in men. ● ● Mental changes. ● ● Muscle pain. ● ● Nausea and vomiting. ● ● Sensitivity to cold. ● ● Unintentional weight loss. ● ● Vision disturbances. ● ● Weakness. MEN II signs and symptoms are related to the gland(s) affected by the malignancy. For example, an affected thyroid gland causes an enlarged thyroid mass, elevated calcitonin, and sometimes, evidence