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[类癌样促肾上腺皮质激素活性胸腺肿瘤伴肾上腺增生及伊滕科-库欣综合征]

[Carcinoid-like ACTH-active thymus tumor with adrenal hyperplasia and the Itsenko-Cushing syndrome].

作者信息

Sidorin V S

出版信息

Arkh Patol. 1984;46(7):58-62.

PMID:6089716
Abstract

Rare carcinoid-like ACTH-active tumour of the thymus in a woman of 40 is described. The tumour was of a solid-trabecular structure with rosette-like figures and consisted of spindle, polyhedral and roundish cells containing electron-dense granules of the neurosecretory type. Hormonal activity of the tumour was manifested in a pronounced hypercorticism with a marked adrenal hyperplasia, atrophy of sex organs and general virilization, skeletal muscle atrophy, osteoporosis, signs of diabetes mellitus, lipid hepatosis and other symptoms of a grave Itsenko-Cushing' syndrome followed by a fever. In spite of the absence of metastasis tumour was characterised by a rapid expansive growth with the compression of neck veins and venous stasis in both mediastinal organs and the tumour itself with the necrosis in its central parts. The cause of death was the thromboembolism of lung arteries from the thrombotic veins of the lower limbs.

摘要

描述了一名40岁女性患有罕见的类癌样促肾上腺皮质激素活性胸腺肿瘤。肿瘤呈实体小梁结构,有玫瑰花结样形态,由含有神经分泌型电子致密颗粒的梭形、多面体形和圆形细胞组成。肿瘤的激素活性表现为明显的皮质醇增多症,伴有显著的肾上腺增生、性器官萎缩和全身男性化、骨骼肌萎缩、骨质疏松、糖尿病迹象、脂质肝病变以及严重的库欣综合征的其他症状,随后出现发热。尽管没有转移,但肿瘤的特点是迅速膨胀性生长,压迫颈静脉,导致纵隔器官和肿瘤本身出现静脉淤滞,中央部分坏死。死亡原因是下肢血栓静脉导致的肺动脉血栓栓塞。

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