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与库欣综合征相关的胰岛细胞瘤。

Pancreatic islet cell tumor associated with Cushing's syndrome.

作者信息

Clark E S, Carney J A

出版信息

Am J Surg Pathol. 1984 Dec;8(12):917-24. doi: 10.1097/00000478-198412000-00004.

DOI:10.1097/00000478-198412000-00004
PMID:6097131
Abstract

Seven female patients with a pancreatic islet cell tumor and Cushing's syndrome are described. Five patients initially had symptoms and signs of hypercortisolism, and two presented with an abdominal mass and had subsequent development of the syndrome. The tumors ranged in size from 2 to 12 cm in diameter, and all were argyrophilic with the Grimelius technique. Four cases had positive immunostaining for adrenocorticotropic hormone; other hormones were identified in four cases. Six of the patients were dead at the time of this report: five with metastatic tumor within 4 years of diagnosis and one 19 years after diagnosis with hepatic metastases of undetermined origin. One patient was alive with hepatic metastases 20 years after operation. Among the 42 known cases of pancreatic islet cell tumor and Cushing's syndrome, the 5-year survival was 16%.

摘要

本文描述了7例患有胰岛细胞瘤和库欣综合征的女性患者。5例患者最初有皮质醇增多症的症状和体征,2例表现为腹部肿块,随后出现该综合征。肿瘤直径为2至12厘米,采用格里梅利乌斯技术染色均呈嗜银性。4例促肾上腺皮质激素免疫染色呈阳性;4例检测到其他激素。撰写本报告时,6例患者已死亡:5例在诊断后4年内出现转移性肿瘤,1例在诊断19年后出现不明来源的肝转移。1例患者术后20年有肝转移但仍存活。在42例已知的胰岛细胞瘤和库欣综合征病例中,5年生存率为16%。

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