垂体促肾上腺皮质激素分泌型癌伴难治性库欣病及肝转移：一例报告并文献复习
ACTH-producing carcinoma of the pituitary with refractory Cushing's Disease and hepatic metastases: a case report and review of the literature.
作者信息
Pinchot Scott N, Sippel Rebecca, Chen Herbert
机构信息
Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, WI, USA.
出版信息
World J Surg Oncol. 2009 Apr 8;7:39. doi: 10.1186/1477-7819-7-39.
BACKGROUND
Pituitary carcinomas are rare neuroendocrine tumors affecting the adenohypophysis. The hallmark of these lesions is the demonstration of distant metastatic spread. To date, few well-documented cases have been reported in the literature.
CASE PRESENTATION
Here, we report the case of a fatal pituitary carcinoma evolving within two years from an adrenocorticotrophic hormone (ACTH)-secreting macroadenoma and review the global literature regarding this rare neuroendocrine tumor.
CONCLUSION
Pituitary carcinomas are extremely rare neoplasms, representing only 0.1% to 0.2% of all pituitary tumors. To date, little is understood about the molecular basis of malignant transformation. The latency period between initial presentation of a pituitary adenoma and the development of distal metastases marking carcinoma is extremely variable, and some patients may live well over 10 years with pituitary carcinoma.
背景
垂体癌是一种罕见的神经内分泌肿瘤，影响腺垂体。这些病变的标志是远处转移扩散的证据。迄今为止，文献中报道的记录良好的病例很少。
病例报告
在此，我们报告一例致命性垂体癌病例，该病例在两年内由分泌促肾上腺皮质激素（ACTH）的大腺瘤演变而来，并回顾了关于这种罕见神经内分泌肿瘤的全球文献。
结论
垂体癌是极其罕见的肿瘤，仅占所有垂体肿瘤的0.1%至0.2%。迄今为止，对恶性转化的分子基础了解甚少。垂体腺瘤初次出现与标志着癌的远处转移发生之间的潜伏期变化极大，一些垂体癌患者可能存活超过10年。
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