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静默型促肾上腺皮质激素细胞腺瘤

Silent corticotroph adenomas.

作者信息

Karavitaki Niki, Ansorge Olaf, Wass John A H

机构信息

Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, UK.

出版信息

Arq Bras Endocrinol Metabol. 2007 Nov;51(8):1314-8. doi: 10.1590/s0004-27302007000800017.

DOI:10.1590/s0004-27302007000800017
PMID:18209869
Abstract

Silent corticotroph pituitary adenomas (SCA) are defined as pituitary adenomas showing positive staining for adrenocorticotrophic hormone in immunohistochemical studies, but not associated with perioperative clinical or laboratory features of hypercortisolaemia. They account for 1.1-6% of surgically removed pituitary adenomas. Currently, two distinct pathologic subtypes of SCA are recognised. Their pathogenesis remains unclear. They present with local mass effects (headache, visual deterioration, cranial nerve palsies, endocrine dysfunction). The lack of manifestations of cortisol excess has not been conclusively explained. In surgical series, most tumours are macroadenomas with suprasellar extension present in 87-100% of the cases; this is in contrast to Cushing's disease, which is mostly attributed to microadenomas. Surgery remains the main therapeutic approach. Attempts to identify predictors of recurrence have not been successful. Management and follow-up protocols should be planned taking into account their potential aggressive behaviour, particularly upon recurrence. The development of florid pituitary Cushing's syndrome and local recurrence followed by metastatic disease (occasionally outside the central nervous system) have been rarely reported.

摘要

静止性促肾上腺皮质激素细胞垂体腺瘤(SCA)被定义为在免疫组织化学研究中显示促肾上腺皮质激素染色阳性,但与围手术期高皮质醇血症的临床或实验室特征无关的垂体腺瘤。它们占手术切除垂体腺瘤的1.1%-6%。目前,已识别出SCA的两种不同病理亚型。其发病机制仍不清楚。它们表现为局部占位效应(头痛、视力减退、颅神经麻痹、内分泌功能障碍)。皮质醇过量表现的缺乏尚未得到确凿解释。在手术系列中,大多数肿瘤为大腺瘤,87%-100%的病例存在鞍上延伸;这与库欣病相反,后者大多归因于微腺瘤。手术仍然是主要的治疗方法。识别复发预测因素的尝试尚未成功。应考虑到它们潜在的侵袭性行为,特别是在复发时,制定管理和随访方案。垂体库欣综合征明显发作以及局部复发后发生转移性疾病(偶尔发生在中枢神经系统外)的情况很少有报道。

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