Pheochromocytoma |
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General principles [1][2][6]
Multidisciplinary care at a specialized center is recommended. Surgical tumor resection is the only curative treatment. Preoperative blood pressure management and postoperative monitoring for adrenal crisis are essential. Management of advanced nonoperable disease (e.g., metastatic disease) is palliative and may include: [7] Cytoreductive therapies (e.g., cytoreductive surgery, radiotherapy, chemotherapy) Symptomatic therapy (e.g., alpha blockers) Patients with germline mutations should be included in surveillance programs led by endocrinologists.Treat hypertensive crisis due to pheochromocytoma with IV antihypertensives that target catecholamine excess (e.g., phentolamine, clevidipine, or nicardipine) and avoid beta blockers. [15][16][17] Surgical resection [1][2][6] Adrenalectomy: treatment of choice for pheochromocytoma Subtotal (organ sparing) adrenalectomy: removal of the part of the gland containing the tumor Total (radical) adrenalectomy: removal of the entire adrenal gland Techniques Laparoscopic resection: preferred for most tumors Open resection: preferred for large; (> 6 cm) or invasive tumors Removal of paragangliomas: Open resection is preferred. [1]A no-touch technique should be used because contact with the tumor can lead to massive catecholamine release. Preoperative management [1][2][7]Preoperative management of blood pressure and heart rate is required. Treatment target BP , > 90 mmHg systolic Heart rate 60–80/min while seated Pharmacotherapy: Initiate 7–14 days prior to surgery. [1] Indicated for all patients with hormonally active pheochromocytoma Start with alpha-adrenergic blockade. Nonselective irreversible: phenoxybenzamine [1][2] OR selective alpha-1 blocker: doxazosin (off label) [1] Add a beta blocker after effective alpha blockade to control tachycardia, e.g.: [2] Metoprolol (off label) OR propranolol Consider the following for additional blood pressure control: Calcium channel blockers, e.g., amlodipine [1] Metyrosine [1] Nonpharmacological therapy: Ensure high sodium and fluid intake to prevent postoperative hypotension. [2]Treat PHEochromocytoma with PHEnoxybenzamine, but remember that Alpha blockers should be given Ahead and Beta blockers Behind. Starting beta blockers before alpha blockers is contraindicated because doing so can cause life-threatening hypertensive crisis. Beta-blockers cancel out the vasodilatory effect of peripheral beta-2 adrenoceptors, potentially leading to unopposed alpha-adrenoceptor stimulation and thereby causing vasoconstriction and increased blood pressure. Postoperative management [1][2][6] Postoperative monitoring Measure blood pressure, heart rate, and blood glucose levels for 24–48 hours. Start management for acute adrenal insufficiency as needed (e.g., glucocorticoids, IV fluids). Long-term monitoring May include repeat biochemical testing and imaging studies; see “Diagnostics.” The frequency of monitoring is based on individual risk for persistent, recurrent, and/or metastatic disease. [7]The risk of postoperative acute adrenal insufficiency and subsequent hypoglycemia is especially high in individuals with bilateral adrenalectomy. |
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