本病系1961年Sipple首先报告,故又名Sipple综合征。MEN—Ⅱ_a型具有常染色体显性遗传性肿瘤三联征:甲状腺髓样癌(MCT)、嗜铬细胞瘤、甲状旁腺增生症。由于MCT具有特征性的组织形态,常与其它内分泌腺腺瘤并发,显示各种内分泌异常、家族倾向性及合并症等,临床上较罕见,国内报告极少,笔者遇见1例,结合文献复习,阐述了MEN—Ⅱ_a的形态特征、组织发生、家族性与合并症、临床表现与诊断及治疗与预后。 The disease was first reported by Sipple in 1961 and it is also known as Sipple syndrome. The MEN-II_a type has an autosomal dominant tumor neoplasm: medullary thyroid carcinoma (MCT), pheochromocytoma, and parathyroid hyperplasia. Because MCT has a characteristic tissue morphology, often with other endocrine adenomas, showing various endocrine abnormalities, familial tendencies, complications, etc., are rare in clinical practice, domestic reports are rare, the author met one case, combined with literature review The morphological features, histogenesis, familial and comorbidities, clinical manifestations and diagnosis, treatment and prognosis of MEN-II_a were described.