MULTIPLE ENDOCRINE NEOPLASIA-2 (MEN-2) MEN-2A (SIPPLE SYNDROME)

被引：11

作者：

KOUSSEFF, BG

机构：

来源：

DERMATOLOGIC CLINICS | 1995年 / 13卷 / 01期

关键词：

D O I：

10.1016/S0733-8635(18)30111-6

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

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.

引用

页码：91 / 97

页数：7