Wollensak G, Herbst E W, Beck A, Schaefer H E
Department of Pathology, University of Freiburg, Federal Republic of Germany.
Virchows Arch A Pathol Anat Histopathol. 1992;420(2):191-5. doi: 10.1007/BF02358812.
In a 52-year-old Caucasian man osteopoikilosis had been misdiagnosed roentgenologically 2 years before his death. Gradually he developed Cushing's syndrome and ultimately superior vena caval obstruction. At autopsy a primary thymic carcinoid with extensive osteoblastic bone metastasis was found. Immunohistochemically the tumor was shown to be positive for adrenocorticotropic hormone (ACTH), cytokeratin (KL1), neuron-specific enolase, synaptophysin, chromogranin and glucagon. Remarkably the tumour was negative for serotonin despite high urinary hydroxyindolacetic acid levels. Bilateral hyperplasia of the adrenal cortex was found. The adenohypophysis showed a considerable reduction of ACTH-producing cells and numerous Crooke's cells with a characteristic immunohistochemical pattern.
一名52岁的白种男性,骨斑点症在其去世前2年被放射学误诊。他逐渐发展为库欣综合征,最终出现上腔静脉阻塞。尸检发现原发性胸腺类癌伴广泛成骨性骨转移。免疫组化显示肿瘤促肾上腺皮质激素(ACTH)、细胞角蛋白(KL1)、神经元特异性烯醇化酶、突触素、嗜铬粒蛋白和胰高血糖素呈阳性。值得注意的是,尽管尿中羟吲哚乙酸水平很高,但肿瘤5-羟色胺呈阴性。发现双侧肾上腺皮质增生。腺垂体促肾上腺皮质激素分泌细胞明显减少,有许多具有特征性免疫组化模式的克鲁克细胞。