Roentgen Technics in the Diagnosis and Localization of Pheochromocytoma

The term pheochromocytoma is Used to designate the relatively rare functioning tumor arising from the chromaffin tissue of the adrenal medulla or from chromaffin tissue elsewhere in the body. Patients may present with either paroxysmal or sustained hypertension and a wide variety of bizarre symptoms. Slightly over 10 per cent of pheochromocytomas are multiple or extra-adrenal. The most frequent sites in such instances are the opposite adrenal, the sympathetic ganglia, and the organs of Zuckerkandl alongside of the terminal portion of the abdominal aorta. Paraganglioma and chromaffinoma are alternate terms used to describe the extra-adrenal tumors, but for the purpose of this discussion all functioning chromaffin tumors, either adrenal or extra-adrenal, producing hypertension and certain associated symptoms will be classified as pheochromocytomas. They are reported occasionally to arise from either normal or ectopic chromaffin tissue in the chest, neck, and bladder (29, 30, 42, 58). Incidence Estimates by different investigators between 1950 and 1961 have suggested the incidence of pheochromocytoma to range between 0.32 and 0.5 per cent of all cases of hypertension (30, 53, 60). Graham, employing Smithwick's incidence of 0.47 per cent and White's estimate of over 100,000 annual deaths in the United States from hypertension, suggests that between 600 and 800 people may die annually in this country from pheochromocytoma (29). Although the first pheochromocytoma was described at autopsy before 1900 (26), a review of literature in 1937 revealed only 27 case reports to that date (67). Slightly over twelve years later Graham was able to locate 280 cases in the world literature (29); by Jan. 1, 1957, Barbeau (3) had collected 626 cases (an additional 346 cases in seven and one-half years). While the actual incidence is still to be definitely established, it is apparent that these hypertension-causing tumors of the adrenal medulla are being discovered and reported with increasing frequency. The dramatic cure usually effected when a pheochromocytoma is removed before irreversible cardiovascular damage has occurred has led almost all clinics interested in hypertension to screen many of their patients by either chemical or pharmacological tests or by assay of the urinary catecholamines (27). Current interest in this surgically curable form of hypertension stems not only from the continued improvement in the accuracy and safety of the diagnostic tests for pheochromocytoma but also from the marked improvement in surgical results achieved during the past two decades. The operative mortality for Graham's 1949 collective review was a high 26 per cent, but the series contained a number of early reported cases, when the nature of the tumor was not well understood. In 1960 the Mayo Clinic group was able to report a personal series of 76 pheochromocytomas in 66 patients.