Once the diagnosis is confirmed by biochemical determinations, the localization and extent of disease should be determined. 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. 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. 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. 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.
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- 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.
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- 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.
- 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.
- Fink IJ, Reinig JW, Dwyer AJ, et al.: MR imaging of pheochromocytomas. J Comput Assist Tomogr 9 (3): 454-8, 1985 May-Jun.
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