多发性内分泌腺肿瘤是一种遗传性疾病,其特点为多种内分泌腺同时发病,其确切的发病率尚不清楚,但并非罕见,近年来由于放射免疫诊断的应用,使该病得以早期诊断。临床分为:Ⅰ型(MEN_I)。Ⅱ型(MEN_(Ⅱa))、Ⅲ型(MEN_(Ⅱb)或MEN_Ⅲ)。复习近期文献,对其病因、病理及各类型的临床表现、诊断、治疗等问题综述如下: 一、病因、病理 1966年Pearse首先提出:产生肽类神经元和肽类激素的细胞,虽然广泛分布于身体的不同部位,但却具有共同的生化特点:它能摄取胺的前体,脱出羧基而变为活性胺(Amine Precursor Uptoke and Decarbo-xylation),这一类细胞简称为APUD细胞。目前认为这类细胞的全部或绝大多数共同来源于外胚层,特别是神经嵴和神经板。在这些结构形成后不久,细胞即移行到身体的其它部位,有的在神经系统之内,成为 Multiple endocrine neoplasia is a hereditary disease characterized by the simultaneous onset of various endocrine glands. The exact incidence is not yet clear, but it is not uncommon. In recent years, the diagnosis of the disease has been diagnosed by radioimmunoassay. . Clinical classification is: Type I (MEN_I). Type II (MEN_(IIa)), Type III (MEN_(IIb) or MEN_III). Reviewing the recent literature, the causes, pathology and various types of clinical manifestations, diagnosis, and treatment are summarized as follows: 1. Etiology, pathology Pearse first proposed in 1966 that cells that produce peptide neurons and peptide hormones are widely distributed In different parts of the body, but has a common biochemical characteristics: it can take up the precursor of amines, remove carboxyl and become active amine (Amine Precursor Uptoke and Decarbo-xylation), this type of cell is abbreviated as APUD cell. It is currently believed that all or most of these cells are derived from the ectoderm, in particular the neural crest and the neural plate. Shortly after these structures are formed, cells migrate to other parts of the body, some within the nervous system, becoming