嗜铬细胞瘤所致异位促肾上腺皮质激素综合征：病例报告及文献复习
Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature.
作者信息
Forman B H, Marban E, Kayne R D, Passarelli N M, Bobrow S N, Livolsi V A, Merino M, Minor M, Farber L R
出版信息
Yale J Biol Med. 1979 Mar-Apr;52(2):181-9.
Abstract
A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure.
摘要
一名51岁女性术前被诊断为分泌促肾上腺皮质激素（ACTH）的嗜铬细胞瘤。该诊断基于她的阵发性高血压、色素沉着和低钾血症。切除右侧肾上腺嗜铬细胞瘤后，血清和尿皮质类固醇、血浆ACTH、尿香草扁桃酸（VMA）和儿茶酚胺水平升高的情况有所下降。随后发现该肿瘤含有高含量的ACTH。文献回顾表明，该综合征的死亡率为57%。术前正确的识别和处理可实现完全治愈。
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