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由多结节性促肾上腺皮质激素细胞增生引起的难治性库欣病。

Refractory Cushing's disease caused by multinodular ACTH-cell hyperplasia.

作者信息

McKeever P E, Koppelman M C, Metcalf D, Quindlen E, Kornblith P L, Strott C A, Howard R, Smith B H

出版信息

J Neuropathol Exp Neurol. 1982 Sep;41(5):490-9. doi: 10.1097/00005072-198209000-00002.

DOI:10.1097/00005072-198209000-00002
PMID:6286892
Abstract

A patient with pituitary-dependent hypercortisolism, unresponsive to resection of nodules in the anterior lobe, is described. Histochemical stains of the nodules showed multiple, focal, cellular expansions of the fibrovascular stroma. Transitions between normal and expanded adenohypophysial acini were present. Immunoperoxidase stains for ACTH and other pituitary hormones revealed that these multiple foci contained an excess of ACTH-positive cells. Less than 10% of the cells in these foci were negative for ACTH and positive for other hormones. Serial sections showed that these foci of predominantly ACTH-producing acini were not connected. Clinical, morphological, and immunohistochemical data indicated that ACTH-cell hyperplasia caused Crushing's disease in this patient. Pathologic study of individual cases should concentrate on determining whether hyperplasia or adenoma exist at the time of surgical exploration of the pituitary gland, since this determination is important to proper treatment. Tentative criteria to recognize ACTH-cell hyperplasia are: 1. Multiple foci of ACTH laden cells. 2. A minor subpopulation of cells of alternate hormone series. 3. Expansion without destruction of acini in the adenohypophysis.

摘要

本文描述了一名垂体依赖性皮质醇增多症患者,该患者对切除前叶结节无反应。结节的组织化学染色显示纤维血管基质有多个局灶性细胞扩张。正常腺垂体腺泡与扩张腺泡之间存在过渡。促肾上腺皮质激素(ACTH)和其他垂体激素的免疫过氧化物酶染色显示,这些多个病灶含有过量的ACTH阳性细胞。这些病灶中不到10%的细胞ACTH阴性而其他激素阳性。连续切片显示,这些主要产生ACTH的腺泡病灶不相连。临床、形态学和免疫组化数据表明,ACTH细胞增生导致了该患者的柯兴氏病。对个别病例的病理研究应集中于在垂体手术探查时确定是否存在增生或腺瘤,因为这一判定对恰当治疗很重要。识别ACTH细胞增生的初步标准为:1. 多个充满ACTH的细胞病灶。2. 少量交替激素系列的细胞亚群。3. 腺垂体腺泡扩张但未破坏。

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