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Once the diagnosis is confirmed by biochemical determinations, the localization and extent of disease should be determined.[9] Ninety-seven percent of the tumors are found in the abdomen, 2% to 3% are found in the thorax, and 1% are found in the neck. The initial studies should be a chest film and abdominal computed tomographic (CT) scan. I131 meta-iodobenzylguanidine (MIBG) has been found to be useful as a scintigraphic localization agent.[14,15] If the tumor is not adequately localized by these methods, then magnetic resonance imaging (MRI), or rarely, vena cava catheterization with selective venous sampling for catecholamines may be indicated.[16] CT and MRI scans are about equally sensitive (98% to 100%), while MIBG scanning has a sensitivity of only 80%. MIBG scanning, however, has a specificity of 100%, compared to a specificity of 70% for CT and MRI.[12] If extra-adrenal or metastatic disease is suspected, additional studies such as bone scan, liver-spleen scan, chest CT scan, or ultrasound may aid in determining the extent of disease.

Surgical resection is the standard curative modality.[17] If the primary tumor is localized to the adrenal gland and is benign, then survival is that of the normal age-matched population. In patients with unresectable, recurrent, or metastatic disease, long-term survival is possible; the overall 5-year survival, however, is less than 50%. Pharmacologic treatment of the catecholamine excess is mandatory and surgery, radiation therapy, or chemotherapy may provide palliative benefit.

References:

  1. 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.
  2. Neumann HP, Berger DP, Sigmund G, et al.: Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med 329 (21): 1531-8, 1993.
  3. Sclafani LM, Woodruff JM, Brennan MF: Extraadrenal retroperitoneal paragangliomas: natural history and response to treatment. Surgery 108 (6): 1124-9; discussion 1129-30, 1990.
  4. Whalen RK, Althausen AF, Daniels GH: Extra-adrenal pheochromocytoma. J Urol 147 (1): 1-10, 1992.
  5. Pommier RF, Vetto JT, Billingsly K, et al.: Comparison of adrenal and extraadrenal pheochromocytomas. Surgery 114 (6): 1160-5; discussion 1165-6, 1993.
  6. Manger WM, Gifford RW: Pheochromocytoma. New York: Springer-Verlag, 1977.
  7. 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.
  8. 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.
  9. Bravo EL, Gifford RW Jr: Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med 311 (20): 1298-303, 1984.
  10. Remine WH, Chong GC, Van Heerden JA, et al.: Current management of pheochromocytoma. Ann Surg 179 (5): 740-8, 1974.
  11. Lenders JW, Keiser HR, Goldstein DS, et al.: Plasma metanephrines in the diagnosis of pheochromocytoma. Ann Intern Med 123 (2): 101-9, 1995.
  12. Bravo EL: Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma. Endocr Rev 15 (3): 356-68, 1994.
  13. Sjoberg RJ, Simcic KJ, Kidd GS: The clonidine suppression test for pheochromocytoma. A review of its utility and pitfalls. Arch Intern Med 152 (6): 1193-7, 1992.
  14. McEwan AJ, Shapiro B, Sisson JC, et al.: Radio-iodobenzylguanidine for the scintigraphic location and therapy of adrenergic tumors. Semin Nucl Med 15 (2): 132-53, 1985.
  15. Shapiro B, Copp JE, Sisson JC, et al.: Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases. J Nucl Med 26 (6): 576-85, 1985.
  16. Fink IJ, Reinig JW, Dwyer AJ, et al.: MR imaging of pheochromocytomas. J Comput Assist Tomogr 9 (3): 454-8, 1985 May-Jun.
  17. Brennan MF, Keiser HR: Persistent and recurrent pheochromocytoma: the role of surgery. World J Surg 6 (4): 397-402, 1982.
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Last Updated: March 09, 2010
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