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胸腺色素沉着性梭形细胞类癌伴异位促肾上腺皮质激素分泌:一种罕见变异型的报告及纵隔梭形细胞肿瘤的鉴别诊断

Pigmented spindle cell carcinoid tumour of the thymus with ectopic adrenocorticotropic hormone secretion: report of a rare variant and differential diagnosis of mediastinal spindle cell neoplasms.

作者信息

Kuo T-t

机构信息

Department of Pathology, Chang Gung University School of Medicine and Chang Gung Memorial Hospital, Kwei San, Tao Yuan, Taiwan.

出版信息

Histopathology. 2002 Feb;40(2):159-65. doi: 10.1046/j.1365-2559.2002.01339.x.

Abstract

AIMS

A variety of histological variants of thymic carcinoid tumour have been described. A rare case of pigmented spindle cell carcinoid tumour of the thymus is documented and compared with the reported cases of thymic pigmented carcinoid tumour in the literature, with a discussion of the differential diagnosis of spindle cell tumours of the mediastinum.

METHODS AND RESULTS

A thymic tumour with ectopic adrenocorticotropic hormone (ACTH) secretion was resected from a 24-year-old man suffering from Cushing's syndrome. Histological, immunohistochemical, and ultrastructural studies revealed an ACTH-producing spindle cell carcinoid tumour harbouring pigmented melanocytes. Among four thymic pigmented carcinoid tumours reported before, only one was similar to the present case by being also an ACTH-secreting pigmented spindle cell thymic carcinoid tumour. The clinicopathological features of this tumour distinguish it from a spindle cell thymoma, spindle cell thymic carcinoma, and other mediastinal spindle cell tumours.

CONCLUSIONS

This case illustrates an extremely rare variant of thymic carcinoid tumour exhibiting a spindle cell morphology and harbouring pigmented melanocytes. Awareness of this histological variant is important in the differential diagnosis of spindle cell tumours of the mediastinum.

摘要

目的

胸腺类癌肿瘤存在多种组织学变异类型。本文记录了一例罕见的胸腺色素性梭形细胞类癌肿瘤,并与文献中报道的胸腺色素性类癌肿瘤病例进行比较,同时探讨纵隔梭形细胞瘤的鉴别诊断。

方法与结果

从一名患有库欣综合征的24岁男性患者身上切除了一个分泌异位促肾上腺皮质激素(ACTH)的胸腺肿瘤。组织学、免疫组织化学和超微结构研究显示,该肿瘤为产生ACTH的梭形细胞类癌肿瘤,伴有色素沉着的黑素细胞。在之前报道的4例胸腺色素性类癌肿瘤中,只有1例与本病例相似,同样是分泌ACTH的色素性梭形细胞胸腺类癌肿瘤。该肿瘤的临床病理特征使其有别于梭形细胞胸腺瘤、梭形细胞胸腺癌及其他纵隔梭形细胞瘤。

结论

本病例展示了胸腺类癌肿瘤一种极其罕见的变异类型,其具有梭形细胞形态并伴有色素沉着的黑素细胞。认识这种组织学变异类型对于纵隔梭形细胞瘤的鉴别诊断具有重要意义。

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