MALIGNANT PHEOCHROMOCYTOMA - A SERIES OF 14 CASES OBSERVED BETWEEN 1966 AND 1990

We report 14 patients (9 males, 5 females) aged 15-59 years, treated for malignant pheochromocytoma. These patients were observed during the 1966-1990 period along with 68 other patients presenting benign pheochromocytomas. From the initial general presentation of the 14 patients, two groups could be individualized. In seven patients, the initial presentation seemed benign. After the excision, the recovery was complete, but patients recurred on average 7.8 yr later (range 1-22 yr). Tumors were intraadrenal in six cases (5 single, 1 bilateral) and extraadrenal in one case. In the seven remaining patients, malignancy was evident from the first examination. The tumors were intraadrenal in 2 cases, extraadrenal in 5 cases. Frequency of extraadrenal locations (6/14) was in this series significantly higher than in benign forms (9/68). Diagnosis of malignancy was based on metastases in 12 cases (lymph nodes in 5, bones in 5, liver in 4, lung in 2, brain in 1) and on peritumoral extension in 2 cases. No biological specificity was detected in urinary excretion of catecholamines or its metabolites. In 6 patients so far studied, an uptake of I-131 MIBG was found in the tumor and/or metastases. Four patients received therapeutic doses of I-131 MIBG and in three of them, this treatment led to a good result within a follow-up range of 12 to 66 months. The general evolution of the eleven cases followed up consisted of 5 patients who deceased with a median of 2 yr (range 1-12 yr) and 6 patients with a long survival 6.5 yr (range 2-15 yr) after the diagnosis of malignancy which corresponds to an overall survival of 14 yr (range 6-24 yr).