Mineur Laurent, Boustany Rania, Vazquez Léa
Institute Sainte Catherine, Gastrointestinal and Liver Cancer Unit, Avignon, France.
Case Rep Oncol. 2021 Sep 23;14(3):1407-1413. doi: 10.1159/000518316. eCollection 2021 Sep-Dec.
Ectopic production of adrenocorticotropic hormone (ACTH) by gastrointestinal neuroendocrine tumours (NETs) is relatively uncommon. We report a rare case of a liver metastatic G1 low-grade NET of the intestine that induced hypercortisolism after surgical resection. A 50-year-old man was admitted for an intestinal obstruction caused by a tumour of the intestine. Paraneoplastic Cushing syndrome was diagnosed more than a year later following the appearance of cushingoid symptoms, despite stable disease according to RECIST criteria but chromogranin A increase. Ketoconazole and sandostatin medical treatment and liver chemoembolization never managed to control the hypercortisolism unlike the bilateral adrenalectomy. The identification and effective management of this uncommon statement of ectopic ACTH secretion is important to improve the patient's prognosis and quality of life.
胃肠道神经内分泌肿瘤（NETs）异位分泌促肾上腺皮质激素（ACTH）相对少见。我们报告一例罕见的肠道G1低级别NET肝转移病例，该病例在手术切除后引发了皮质醇增多症。一名50岁男性因肠道肿瘤导致肠梗阻入院。在出现库欣样症状一年多后，尽管根据RECIST标准疾病稳定但嗜铬粒蛋白A升高，仍诊断为副肿瘤性库欣综合征。与双侧肾上腺切除术不同，酮康唑、善龙药物治疗及肝动脉化疗栓塞均未能控制皮质醇增多症。识别并有效处理这种罕见的异位ACTH分泌情况对于改善患者预后及生活质量至关重要。
