THE PHEOCHROMOCYTOMA - A BENIGN, INTRA-ADRENAL, HYPERTENSIVE, SPORADIC UNILATERAL TUMOR - DOES IT EXIST

This study aims to examine the frequency of the pheochromocytoma (pheo ), defined as a ''benign, intra-adrenal, hypertensive, sporadic, unila teral tumor.'' Three large series amounting to 310 subphrenic chromaff in tumors operated over periods of 17, 23, and 41 years, respectively, have been reviewed. Among those combined 310 pheos, 48 (15.5%) were m alignant (i.e., metastatic) and 262 (84.5%) were benign; 42 (13.5%) we re ectopic (35% of malignant tumors versus 9.5% of benign tumors); 230 (74.2%) were hypertensive (74% of benign tumors versus 73% of maligna nt tumors); 29 (9.4%) were bilateral, including 23 patients with a fam ily history; 41 (13.2%) of patients had MEN II type A or B syndrome; a nd 20 (6.5%) occurred in a phacomatosis setting. Some of the patients had a non-MEN family history of pheo (n = 5), seemingly sporadic hyper parathyroidism (n = 4), or other associated neuroendocrine tumor (n = 9). Sometimes several of these features were combined. Finally 125 (40 .3%) cases fitted the classic description of the tumor, 47.0% at the t ime of initial presentation and 40.3% at the end of follow-up. Late oc currence of metastases or metachronous diagnosis of familial disease m ake lifelong follow-up mandatory. Genetic studies may be indicated in pheochromocytoma patients.