Objectives: Multiple endocrine neoplasia type 1 (MEN1) mainly affects
parathyroid glands, pancreatic islets and pituitary gland. The aim of
this study in 95 MEN1 patients was to examine less frequent localizati
ons: thymic and bronchic neuroendocrine tumors (NET). Patients and Met
hods: Two cases of bronchic NET were observed, both in women, and 4 ca
ses of thymic NET, all in men, giving a prevalence of 7.3%. These NET
were often asymptomatic. A metastatic diffusion was observed in 3 case
s. Elevated plasma levels of glycoprotein hormone alpha subunit (SU) a
nd FSH were observed in 3 and 1 cases respectively. Immunohistochemist
ry indicated the tumor cells to be frequently positively stained for H
CGalpha and FSH. Discussion: We conclude that all patients with MEN1 s
hould undergo screening for thoracic NET, especially in high risk fami
lial subgroups and in case of elevated plasma alphaSU or FSH.