Malignant endocrine tumors in patients with MEN 1 disease

BACKGROUND: In contrast with multiple endocrine neoplasia type 2, malignancies are of minor importance in multiple endocrine neoplasia type 1 (MEN 1) syndrome. METHODS: The data for 42 patients with MEN 1 syndrome were evaluated. Twelve patients (29%) had 1 or more associated malignancies: malignant gastrinoma (1 patient), neuroendocrine tumors of the thymus (2 patients), neuroendocrine tumors of the lung (3 patients), neuroendocrine tumor of the ileum (1 patient), adrenocortical carcinomas (2 patients), and a combination of neuroendocrine tumors of thymus, lung or pancreas (3 patients). RESULTS: Despite suspected MEN 1 syndrome in 7 patients, malignancies were detected late in 4 patients and could not be resected curatively. The survival rates for 5 years and 10 years after operation of the malignant tumor were 66% +/- 14% (SE) and 33% +/- 15% (SE), respectively. CONCLUSIONS: In patients at risk for the MEN 1 syndrome, genetic screening is indispensable. Gene carriers have to be followed up closely with hormone analysis and routine examination of the thorax, pancreas, and adrenal glands to detect malignancies as early as possible.