Abstract
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited disease characterized by parathyroid hyperplasia, pancreatico-gastroduodenal neuroendocrine tumor (NET), pituitary adenoma, adrenal hyperplasia and thymic NET. The causative gene for MEN1 is MEN1 tumor suppressor gene. Multiple endocrine neoplasia type 2 (MEN2) is also an autosomal dominantly inherited disease characterized by medullary thyroid carcinoma (MTC), adrenal pheochromocytoma and parathyroid hyperplasia. The causative gene for MEN2 is RET oncogene. Prognosis of MEN patients is mainly relied on the presence or absence of progressive/metastatic pancreatic NET or MTC. The effective treatment for these tumors had not been well established. In this paper, we described current situation of genetic diagnosis and new promising drug therapy, everolimus, sunitinib and vandetanib for patients with multiple endocrine neoplasia (MEN) in Japan.
