To review the series of patients with pheochromocytoma diagnosed in this institution from 1976 to 1990.
The main clinical features and presentation of 21 patients (13 women and 8 males) with mean age 40 +/- 13 years were reviewed. Urinary excretion of vanillylmandelic acid (VMA) and total catecholamines had been measured. Abdominal echography, computed tomography (CT), adrenal scintigraphy and magnetic resonance (MR) had been used as localizing techniques.
The main clinical feature was hypertension (86%), which was paroxysmal in 57% of patients. 9% were totally asymptomatic at the time of diagnosis. The following diseases were associated: thyroid medullary carcinoma (TMC) in one case, diabetes mellitus (5), cholelithiasis (3), contralateral nonfunctioning adrenal adenoma (2), and renal artery stenosis (1). The values of urinary catecholamines were high in all cases, while that of VMA was falsely negative in 33%. Echography localized the tumor in 10 of the 13 cases where it was carried out, and so did CT in 15 out of 15, scintigraphy in 6 out of 9 and MR in 5 out of 5.
Hypertension, either paroxysmal or not, and increased perspiration were the most remarkable clinical features. VMA measurement has a small diagnostic value. Among the localizing techniques that were used, CT and MR had the highest yield.
[Show abstract][Hide abstract] ABSTRACT: To study distinctive clinical features of pheochromocytoma in the setting of multiple endocrine neoplasia type 2A (MEN 2A) in comparison with sporadic pheochromocytoma.
All patients diagnosed of sporadic pheochromocytoma (n = 29) and in the setting of MEN 2A (n = 16) between 1976 and 1998 in a tertiary hospital were included in the study. The following variables were compared: age at diagnosis, symptoms, presence and characteristics of hypertension, size of the tumor, localization and malignancy. The accuracy of diagnostic tests was also evaluated.
The pheochromocytoma associated to MEN 2A had a higher prevalence in our study (35.5%) and the diagnosis was performed earlier than for sporadic pheochromocytoma (29.1 [7.8] vs. 47,5 [10. 9] years; p < 0.001). Other distinctive features in comparison with sporadic pheochromocytoma were: bilaterality (81.25 vs. 3.44%; p < 0. 001), absence of symptoms (44 vs 11%; p < 0.05) and paroxysmal hypertension. Concerning the diagnostic tests, it must be noted the low sensitivity (60%) of vanillylmandelic acid in MEN 2A.
The pheochromocytoma in the setting of MEN 2A is diagnosed at younger age and frequently asymptomatic and bilateral. In view of the high prevalence of MEN 2A in our country, a genetic analysis should be performed in all patients with pheochromocytoma, especially when it is bilateral.
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