摘要
多发性内分泌瘤病(MEN)的特点是在同一患者身上出现2种或2种以上的内分泌腺体肿瘤。此病以往被称为“多发性内分泌腺病”或“多腺性综合征”,现在命名为MEN。MEN有2种主要形式,1型(MEN1型,Wermer's综合征)和2型(MEN2型,Sipple’s综合征);两型各自有其特异的内分泌腺体肿瘤生成(表1)。MEN综合征的临床表现与肿瘤的部位及其分泌产物有关。对每种肿瘤的诊断和治疗与非MEN患者的相类似。
Multiple endocrine neoplasia (MEN) is characterized by tumours involving two or more endocrine glands within a single patient. This disorder has previously been termed 'multiple endocrine adenopathy' and 'pluriglandular syndrome', but MEN is now the preferred term. There are two major forms of MEN, type 1 (MEN1, Wermer's syndrome) and type 2 (MEN2, Sipple's syndrome); each form is characterized by the development of tumours within specific endocrine glands (Figure 1). The clinical manifestations of the MEN syndromes are related to the sites of the tumours and to their products of secretion. The diagnosis and management of each tumour is similar to that in non-MEN patients.