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多发性内分泌性腺瘤病(MEA),系指在脑垂体前叶、甲状旁腺、胰岛及更少见者如肾上腺皮质、甲状腺和卵巢,同时产生的腺瘤样病变。卓-艾氏综合征(Z-E Syndrome)即属此类。为了与另一组多发性内分泌性腺瘤病相区别,将其称为多发性内分泌性腺瘤病Ⅰ型(MEA-Ⅰ)。另一类家族性的多发内分泌腺瘤病,包括甲状腺髓样癌、嗜铬细胞瘤(常常是双侧性)、甲状旁腺增生以及舌唇的多发性神经瘤,这一类综合征称为多发性内分泌性腺瘤病Ⅱ型(MEA-Ⅱ),亦称Sipple氏综合征。多发性内分泌性腺瘤病Ⅱa型(MEA-Ⅱa)包括甲状腺髓样癌;嗜铬细胞瘤(常常多发);经常有甲状旁腺增生。多发性内分泌性腺瘤病Ⅱb型(MEA-Ⅱb)包括甲状腺髓样癌;舌或唇的神经瘤;常有嗜铬细胞瘤;马凡氏样表现(marfanoid appearance);甲状旁腺正常。
Multiple endocrine adenomatosis (MEA) refers to an adenomatoid lesion that occurs at the same time in the anterior pituitary, parathyroid gland, islets, and less common ones such as the adrenal cortex, thyroid, and ovary. Z-E Syndrome is this type. In order to distinguish it from another group of multiple endocrine adenomas, it is referred to as multiple endocrine adenomatosis type I (MEA-I). Another type of familial multiple endocrine neoplasia, including medullary thyroid carcinoma, pheochromocytoma (often bilateral), parathyroid hyperplasia, and multiple neuroma of the lips, this type of syndrome is known as Multiple Endocrine Adenomatosis Type II (MEA-II), also known as Sipple’s syndrome. Multiple endocrine neoplasia type IIa (MEA-IIa) includes medullary thyroid carcinoma; pheochromocytoma (often multiple); often hyperparathyroidism. Multiple endocrine neoplasia type IIb (MEA-IIb) includes medullary thyroid carcinoma; neuroma of the tongue or lip; pheochromocytoma often; marfanoid appearance; normal parathyroid gland.