Pheochromocytoma in multiple endocrine neoplasia type 2: European study

Publisher Website
Google Scholar
Abstract
Objectives. Pheochromocytoma (pheo) is the second component of the multiple endocrine neoplasia type 2 (MEN 2) syndrome. Clinical expression is sometimes poor, and chronology between medullary thyroid carcinoma (MTC) and pheo is not well evaluated. Therefore, a retrospective study was done in eight European countries in order to precise the main characteristics of pheo in MEN 2. Subjects. Data from 300 MEN 2 patients with pheo (274 MEN 2 A and 26 MEN 2 B) were obtained from cases registered by the EuroMen study group, and collected by a medical standardized questionnaire. These cases occurred between 1969 and 1992. Results. Mean age at diagnosis of pheo was 39.5 years (range 14–68 years) in MEN 2A and 32.4 years (range 15–41 years) in MEN 2B patients. Pheo occurred first in 25.1% of the cases (2–15 years before diagnosis of MTC) and after MTC in 40.2% (2–11 years). In other cases (34.7%), MTC and pheo were diagnosed at the same time. Involvement was bilateral in 67.8% of cases. Malignancy was only 4%. Thirty‐nine deaths occurred in these 300 patients, 64.1% were linked in pheo, 23.1% to MTC and 12.8% to other causes. Surgery was unilateral in 39.7% of the cases and bilateral adrenalectomy was the first procedure in 48.4%. A bilateral adrenalectomy in two steps had to be done in 11.9% of cases. In conclusion, these results justify systematic and prolonged biochemical screening of pheo during follow‐up of MTC and address some questions about the best mode of surgery.