Useinova Z T, Pigarova E A, Bel'tsevich D G, Chevais A, Dzeranova L K, Sitkin I I, Tarbaeva N V, Khairieva A V, Degtyarev M V, Platonova N M, Troshina E A, Bondarenko E V
Endocrinology Research Centre.
Probl Endokrinol (Mosk). 2023 Nov 2;70(3):55-66. doi: 10.14341/probl13374.
Endogenous hypercorticism (EH) is a severe symptom complex caused by hypercortisolemia; according to the etiology, ACTH-dependent and ACTH-independent variants are distinguished, which, according to the literature, occur in 70-80% and 20-30% of cases, respectively. A rare cause of ACTH-dependent endogenous hypercorticism is ACTH-ectopic syndrome (ACTH-ES) (about 15-20% of cases). ACTH-ES is a syndrome of adrenocorticotropic hormone (ACTH) hyperproduction by neuroendocrine tumors of extrahypophyseal origin. Various tumors can secrete ACTH: bronchopulmonary carcinoid, small cell lung cancer, less frequently, thymus carcinoid, islet cell tumors and pancreatic carcinoid, medullary thyroid cancer, carcinoid tumors of the intestine, ovaries, as well as pheochromocytoma (PCC).This publication presents a clinical case of rarely detected paraneoplastic ACTH production by pheochromocytoma. The patient had clinical manifestations of hypercorticism, therefore, she applied to the Russian National Research Center of Endocrinology of the Ministry of Health of Russia. During the examination Cushing's syndrome (CS) was confirmed, multispiral computed tomography (MSCT) of the abdominal cavity revealed a voluminous formation of the left adrenal gland. Additional examination recorded a multiple increase in urinary catecholamine levels. Subsequently, the patient underwent left-sided adrenalectomy. The diagnosis of pheochromocytoma was confirmed morphologically, immunohistochemical study demonstrated intensive expression of chromogranin A and ACTH by tumor cells.
内源性皮质醇增多症（EH）是一种由高皮质醇血症引起的严重症状复合体；根据病因，可分为促肾上腺皮质激素（ACTH）依赖性和ACTH非依赖性变体，据文献报道，它们分别占病例的70 - 80%和20 - 30%。ACTH依赖性内源性皮质醇增多症的一种罕见病因是ACTH异位综合征（ACTH - ES）（约占病例的15 - 20%）。ACTH - ES是一种由垂体外起源的神经内分泌肿瘤过度产生促肾上腺皮质激素（ACTH）的综合征。各种肿瘤均可分泌ACTH：支气管肺类癌、小细胞肺癌，较少见的有胸腺类癌、胰岛细胞瘤和胰腺类癌、甲状腺髓样癌、肠道类癌、卵巢类癌，以及嗜铬细胞瘤（PCC）。本出版物介绍了一例罕见的嗜铬细胞瘤副肿瘤性ACTH分泌的临床病例。该患者有皮质醇增多症的临床表现，因此，她前往俄罗斯卫生部国家内分泌研究中心就诊。检查期间确诊为库欣综合征（CS），腹腔多排螺旋计算机断层扫描（MSCT）显示左肾上腺有一个巨大肿物。进一步检查发现尿儿茶酚胺水平多次升高。随后，患者接受了左侧肾上腺切除术。形态学确诊为嗜铬细胞瘤，免疫组化研究显示肿瘤细胞强烈表达嗜铬粒蛋白A和ACTH。
