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临床病例研讨会:淋巴细胞性垂体炎:临床病理表现

Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings.

作者信息

Thodou E, Asa S L, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S

机构信息

Department of Pathology, Mount Sinai Hospital, Ontario, Canada.

出版信息

J Clin Endocrinol Metab. 1995 Aug;80(8):2302-11. doi: 10.1210/jcem.80.8.7629223.

DOI:10.1210/jcem.80.8.7629223
PMID:7629223
Abstract

This report describes the clinicopathological features of 16 patients with lymphocytic hypophysitis and compares the results with the published literature. There were 2 males and 14 females in this series. In 10 of the 14 females (71%), the presentation was associated with pregnancy. Nine patients (56%) presented with symptoms of an expanding pituitary sellar mass, 10 (63%) had anterior pituitary hypofunction, 3 had diabetes insipidus (19%). Progressive undiagnosed hypopituitarism led to the demise of 3 patients (19%). Hyperprolactinemia was encountered in 6 patients (38%), and elevated growth hormone levels (GH) resulted in IGF-1 excess in one patient. Computed tomography (CT) and magnetic resonance (MR) imaging revealed features of a pituitary mass mimicking an adenoma in 10 cases (83%). Four patients (25%) had associated autoimmune thyroiditis. Morphologic examination of the pituitary and immunohistochemistry showed a polyclonal lymphoplasmacytic infiltrate as well as occasional neutrophils, eosinophils, and macrophages; the chronic inflammatory process resulted in focal or diffuse adenohypophysial destruction of variable severity with associated fibrosis. The inflammatory infiltrate involved the neurohypophysis in 2 cases and one of these patients had diabetes insipidus; the posterior lobe of two other patients with diabetes insipidus was not examined morphologically. We conclude that lymphocytic hypophysitis should be considered in the differential diagnosis of females with pituitary enlargement presenting in the peripartum period as well as those patients in whom pituitary hormone deficiency and/or excess is noted in association with a co-existing autoimmune disorder. This clinical suspicion should probably also be extended to include patients presenting with rapidly growing pituitary masses associated with compressive symptoms with or without pituitary hormone dysfunction. Because of the transient endocrine and compressive features of this condition in many instances, conservative treatment on the basis of clinical suspicion alone may obviate the need for aggressive pituitary surgery.

摘要

本报告描述了16例淋巴细胞性垂体炎患者的临床病理特征,并将结果与已发表的文献进行比较。该系列中有2例男性和14例女性。14例女性中有10例(71%)发病与妊娠有关。9例患者(56%)表现为垂体窝肿块扩大的症状,10例(63%)有垂体前叶功能减退,3例有尿崩症(19%)。3例患者(19%)因进行性未确诊的垂体功能减退而死亡。6例患者(38%)出现高催乳素血症,1例患者生长激素水平升高导致胰岛素样生长因子-1过量。计算机断层扫描(CT)和磁共振成像(MR)显示10例(83%)有类似腺瘤的垂体肿块特征。4例患者(25%)合并自身免疫性甲状腺炎。垂体形态学检查及免疫组化显示多克隆淋巴细胞浆细胞浸润,偶见中性粒细胞、嗜酸性粒细胞和巨噬细胞;慢性炎症过程导致不同程度的局灶性或弥漫性腺垂体破坏,并伴有纤维化。2例炎症浸润累及神经垂体,其中1例有尿崩症;另外2例有尿崩症的患者未对垂体后叶进行形态学检查。我们得出结论,对于围产期出现垂体肿大的女性以及发现垂体激素缺乏和/或过量并伴有自身免疫性疾病的患者,鉴别诊断时应考虑淋巴细胞性垂体炎。这种临床怀疑可能还应扩大到包括那些出现与压迫症状相关的快速生长的垂体肿块且伴有或不伴有垂体激素功能障碍的患者。由于在许多情况下该病具有短暂的内分泌和压迫特征,仅基于临床怀疑的保守治疗可能无需进行积极的垂体手术。

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