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[胸腺类癌伴异位促肾上腺皮质激素综合征]

[Thymic carcinoid associated with ectopic ACTH syndrome].

作者信息

Ishikawa T, Inoue C, Sasaki H, Satou K, Kimura N

机构信息

Department of Internal Medicine, Sendai Red Cross Hospital, Japan.

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1996 Apr;34(4):471-6.

PMID:8691671
Abstract

A 63-year-old man was admitted to Sendai Red Cross Hospital complaining of chest and back pain associated with Cushing's syndrome. Based on the abnormally high levels of ACTH, cortisol, and CRH in plasma the patient was suspected of having ectopic ACTH syndrome. Histological examination of an extirpated rib and pleural tumor led to the diagnosis of atypical carcinoid tumor, with ribbon and festoon formation, immunoreactivity to ACTH, NSE, Chg-A, and argyrophilia in the tumor cells. Anti-cancer chemotherapy was not effective, and the patient died within a year after the onset of Cushing's syndrome. An autopsy revealed that the patient had an ACTH- and CRH-producing thymic carcinoid with metastases to many organs. The pituitary was atrophic with Crooke's hyaline change. There were many CRH-positive cells in the paraventricular nuclei of the hypothalamus, where no remarkable pathologic changes were seen.

摘要

一名63岁男性因库欣综合征伴胸痛和背痛入住仙台红十字医院。根据血浆中促肾上腺皮质激素(ACTH)、皮质醇和促肾上腺皮质激素释放激素(CRH)水平异常升高,怀疑该患者患有异位ACTH综合征。对切除的肋骨和胸膜肿瘤进行组织学检查,诊断为非典型类癌肿瘤,肿瘤细胞呈带状和花彩状形成,对ACTH、神经元特异性烯醇化酶(NSE)、嗜铬粒蛋白A(Chg-A)免疫反应阳性且嗜银。抗癌化疗无效,患者在库欣综合征发病后一年内死亡。尸检显示患者患有产生ACTH和CRH的胸腺类癌,并伴有多器官转移。垂体萎缩,有克鲁克透明变性。下丘脑室旁核中有许多CRH阳性细胞,未见明显病理变化。

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