Localized Benign Pheochromocytoma
STANDARD TREATMENT OPTIONS:
For localized pheochromocytoma confined to one or both adrenal glands, adequate treatment is complete surgical resection consisting of total adrenalectomy. Although patients with an associated MEN type 2 syndrome are at risk for bilateral tumors, the use of prophylactic contralateral adrenalectomy is not recommended for patients with unilateral tumors.[1] Preoperative medical management of the effect of excessive adrenergic stimulation is necessary. Intraoperatively, blood pressure is controlled by titration of small doses of phentolamine or nitroprusside, and cardiac arrhythmias may be treated with propranolol or lidocaine. Postoperatively, hypotension is common and should be treated with volume expansion (often large volumes are required). Postoperative hypertension is best treated with diuretics. The standard anterior approach to the adrenal gland is used, and the entire gland is removed; simple tumor excision should not be attempted. The abdomen and retroperitoneum are examined thoroughly for the presence of extra-adrenal disease. After one week or more following surgery, repeated biochemical assays for catecholamines and/or metabolites are performed to confirm that all functioning pheochromocytoma has been removed. In experienced hands, the operative mortality is less than 2% to 3% and the end result of complete surgical resection of benign pheochromocytoma is a normal life expectancy.[2,3,4]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized benign pheochromocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References:
- Lairmore TC, Ball DW, Baylin SB, et al.: Management of pheochromocytomas in patients with multiple endocrine neoplasia type 2 syndromes. Ann Surg 217 (6): 595-601; discussion 601-3, 1993.
- Norton JA: Adrenal tumors. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 1528-39.
- Young JB, Landsberg L: Catecholamines and the adrenal medulla: pheochromocytoma. In: Wilson JD, Foster DW, Kronenberg HM, et al., eds.: Williams Textbook of Endocrinology. 9th ed. Philadelphia, Pa: W.B. Saunders Company, 1998, pp 705-716.
- Bravo EL, Gifford RW Jr: Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med 311 (20): 1298-303, 1984.
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