Kato Akihisa, Hayashi Kazuki, Naitoh Itaru, Seno Kyoji, Okada Yukiko, Ban Tesshin, Kondo Hiromu, Nishi Yuji, Umemura Shuichiro, Hori Yasuki, Natsume Makoto, Joh Takashi
Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya 467-8601, Japan.
Department of Gastroenterology, Nagoya West Medical Center, Nagoya 462-8508, Japan.
Mol Clin Oncol. 2016 Jul;5(1):113-116. doi: 10.3892/mco.2016.869. Epub 2016 Apr 21.
Ectopic adrenocorticotropic hormone (ACTH) is rarely secreted by neuroendocrine tumors. Although neuroendocrine tumors may occur at any site in the gastrointestinal system, they very rarely occur in the ampulla of Vater and have a poor prognosis. The present study described the first Cushing's syndrome as a result of ectopic ACTH arising from the ampulla of Vater neuroendocrine carcinoma. A 69-year-old female was admitted with clinical features of Cushing's syndrome, confirmed biochemically by hypokalemia, and elevated levels of ACTH and cortisol. In further investigations, a tumor of the ampulla of Vater and liver metastases were detected. Pathological analysis of the biopsy confirmed a neuroendocrine carcinoma, which was immunohistochemically positive for chromogranin A, synaptophysin, cluster of differentiation 56 and ACTH. Therefore, the present study diagnosed a functional and metastatic neuroendocrine carcinoma of the ampulla of Vater with ectopic ACTH production causing Cushing's syndrome. The patient succumbed to mortality 4 months later, despite administration of combined chemotherapy with irinotecan and cisplatin.
异位促肾上腺皮质激素（ACTH）很少由神经内分泌肿瘤分泌。尽管神经内分泌肿瘤可能发生在胃肠道系统的任何部位，但它们很少发生在 Vater 壶腹，且预后较差。本研究描述了首例因 Vater 壶腹神经内分泌癌产生异位 ACTH 导致的库欣综合征。一名 69 岁女性因库欣综合征的临床特征入院，生化检查证实为低钾血症，且 ACTH 和皮质醇水平升高。在进一步检查中，发现了 Vater 壶腹肿瘤和肝转移。活检的病理分析证实为神经内分泌癌，免疫组化显示嗜铬粒蛋白 A、突触素、分化簇 56 和 ACTH 呈阳性。因此，本研究诊断为功能性和转移性 Vater 壶腹神经内分泌癌，伴有异位 ACTH 分泌导致库欣综合征。尽管给予了伊立替康和顺铂联合化疗，但患者在 4 个月后死亡。
