一例胆囊神经内分泌癌并发异位促肾上腺皮质激素综合征,因败血症导致胎儿迅速死亡。

IF 0.8 Q4 GASTROENTEROLOGY & HEPATOLOGY Clinical Journal of Gastroenterology Pub Date : 2024-08-01 Epub Date: 2024-05-21 DOI:10.1007/s12328-024-01986-z
Kentaro Sato, Tomohiro Suzuki, Kazuki Akaike, Daiki Uchihara, Osamu Ichii, Mayumi Tai, Tadayuki Takagi, Hando Hakozaki, Yutaka Ejiri
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引用次数: 0

摘要

一名 52 岁的女性因主诉上腹胀痛和小腿水肿来我院就诊。计算机断层扫描(CT)显示其肝脏有胆囊肿瘤转移。活检后确诊为神经内分泌癌(NEC)。体格检查显示患者有月牙面容。血液化验显示患者患有低钾血症,肾上腺皮质激素(ACTH)和皮质醇水平较高。地塞米松抑制试验显示皮质醇分泌未被抑制,患者被诊断为胆囊NEC和异位促肾上腺皮质激素综合征(EAS)。为抑制皮质醇分泌,医生给她注射了甲替拉酮,但她因小腿蜂窝组织炎而出现脓毒性休克,于入院第16天死亡。病理解剖显示,死因是弥散性血管内凝血和急性呼吸窘迫综合征。关于胆囊引起的 NEC 导致的 EAS,目前仅有少数病例报道。本文报告的患者在确诊后不久就死亡了,这凸显了治疗胆囊NEC并发EAS所面临的挑战。
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A case of gallbladder neuroendocrine carcinoma complicated by ectopic adrenocorticotropic hormone syndrome and resulting in rapid fetal outcomes due to sepsis.

A 52-year-old woman presented to our hospital with chief complaints of upper abdominal bloating and lower leg edema. Computed tomography (CT) revealed liver metastasis from a gallbladder tumor. This tumor was diagnosed as neuroendocrine carcinoma (NEC) on performing a biopsy. Physical examination revealed a moon face. Blood tests revealed hypokalemia and high levels of adrenocorticotropic hormone (ACTH) and cortisol. Dexamethasone suppression test revealed that cortisol secretion was not suppressed, and the patient was diagnosed with gallbladder NEC and ectopic ACTH syndrome (EAS). Metyrapone was administered to suppress cortisol production; however, she developed septic shock due to cellulitis in the lower leg and died on the 16th day of admission. A pathological autopsy was performed, which revealed disseminated intravascular coagulation and acute respiratory distress syndrome as the cause of death. Only a few cases of EAS due to NEC originating from the gallbladder have been reported. The patient reported here succumbed shortly after diagnosis, thereby highlighting the challenges in treating gallbladder NEC complicated by EAS.

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来源期刊
Clinical Journal of Gastroenterology
Clinical Journal of Gastroenterology GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
2.00
自引率
0.00%
发文量
182
期刊介绍: The journal publishes Case Reports and Clinical Reviews on all aspects of the digestive tract, liver, biliary tract, and pancreas. Critical Case Reports that show originality or have educational implications for diagnosis and treatment are especially encouraged for submission. Personal reviews of clinical gastroenterology are also welcomed. The journal aims for quick publication of such critical Case Reports and Clinical Reviews.
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