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多发性内分泌腺瘤病1型综合征中的垂体腺瘤

Pituitary adenomas of the multiple endocrine neoplasia type I syndrome.

作者信息

Scheithauer B W, Laws E R, Kovacs K, Horvath E, Randall R V, Carney J A

机构信息

Department of Pathology, Mayo Clinic, Rochester, MN 55905.

出版信息

Semin Diagn Pathol. 1987 Aug;4(3):205-11.

PMID:2890193
Abstract

In a series of 1,500 pituitary adenomas surgically resected at Mayo Clinic, 41 (2.7%) occurred in the setting of multiple endocrine neoplasia, type I (MEN-I). Of the 40 patients (18 males, 22 females), 21 (52%) presented with clinical evidence of a pituitary neoplasm, 13 with hyperparathyroidism, and two with functional islet cell tumor. Of the 41 tumors, 11 (27%) were microadenomas, and 30 (73%) were macroadenomas. Immunocytochemical studies demonstrated the following reactivities: GH (4), GH/PRL (6), GH/PRL/glycoprotein (7), GH/ACTH/glycoprotein (1), PRL (16), PRL/TSH (1), ACTH (3), and null cell adenoma (3). We conclude that, in comparison with pituitary adenomas occurring in the general population, those occurring in association with MEN-I are (1) more often endocrinologically functional, (2) more frequently GH- or PRL-producing, and (3) clinicopathologically similar in terms of the subjects age and sex as well as of tumor size and invasiveness.

摘要

在梅奥诊所手术切除的1500例垂体腺瘤系列病例中,41例(2.7%)发生于1型多发性内分泌腺瘤病(MEN - I)背景下。在这40例患者(18例男性,22例女性)中,21例(52%)有垂体肿瘤的临床证据,13例有甲状旁腺功能亢进,2例有功能性胰岛细胞瘤。在这41个肿瘤中,11例(27%)为微腺瘤,30例(73%)为大腺瘤。免疫细胞化学研究显示了以下反应性:生长激素(GH)(4例)、生长激素/催乳素(PRL)(6例)、生长激素/催乳素/糖蛋白(7例)、生长激素/促肾上腺皮质激素(ACTH)/糖蛋白(1例)、催乳素(16例)、催乳素/促甲状腺激素(TSH)(1例)、促肾上腺皮质激素(3例)和无功能细胞腺瘤(3例)。我们得出结论,与一般人群中发生的垂体腺瘤相比,与MEN - I相关的垂体腺瘤(1)内分泌功能更常见,(2)产生生长激素或催乳素的频率更高,(3)在患者年龄、性别以及肿瘤大小和侵袭性方面临床病理相似。

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