Kousseff B G
Division of Medical Genetics, University of South Florida, Tampa, USA.
Dermatol Clin. 1995 Jan;13(1):91-7.
Multiple endocrine neoplasia type 2A (MEN 2A, Sipple syndrome) is an autosomal dominant phakomatosis and is most likely a paracrinopathy. The cardinal manifestations of MEN 2A--medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism--indicate that the condition is one of the inherited cancer syndromes. Cutaneous, lichen amyloidosis-like lesions place MEN 2A among the genodermatoses. The gene of MEN 2A, designated as MEN2A, is in the pericentromeric region of chromosome 10; this allows for reliable prenatal and presymptomatic DNA diagnosis.
2A型多发性内分泌腺瘤病（MEN 2A，西普尔综合征）是一种常染色体显性遗传性错构瘤病，很可能是一种旁分泌病。MEN 2A的主要表现——甲状腺髓样癌、嗜铬细胞瘤和甲状旁腺功能亢进——表明该病是遗传性癌症综合征之一。皮肤苔藓样淀粉样变样病变使MEN 2A属于遗传性皮肤病范畴。MEN 2A的基因，命名为MEN2A，位于10号染色体的着丝粒周围区域；这使得可靠的产前和症状前DNA诊断成为可能。
