Lee Michelle N, Wan WingYee, Chormanski Dianna C, Kravchenko Maria I
Department of Medicine, Department of Endocrinology, San Antonio Military Medical Center, 3551 Roger Brooke Drive, JBSA Fort Sam Houston, San Antonio, TX 78234, USA.
Department of Pathology, San Antonio Military Medical Center, 3551 Roger Brooke Drive, JBSA Fort Sam Houston, San Antonio, TX 78234, USA.
Case Rep Endocrinol. 2020 Feb 26;2020:4869467. doi: 10.1155/2020/4869467. eCollection 2020.
. Pheochromocytomas are rare neuroendocrine tumors that arise from sympathetic adrenomedullary chromaffin tissue. Depending on the amount of catecholamines they secrete, they have variable presentations. There have been reported cases of adrenocorticotrophic (ACTH) secreting pheochromocytomas that present with severe Cushing syndrome. Here, we present a pheochromocytoma with adrenocorticotrophic hormone (ACTH) cosecretion, which due to its rarity and variable presentation, may be a diagnostic challenge. . A 64-year-old woman with history of colon cancer presented with new-onset diabetes, worsening hot flashes, and hypertension. On CT imaging she had an enlarging right adrenal nodule (1.7 cm) with 60 Hounsfield units of attenuation and no PET avidity. Biochemical evaluation showed elevated urinary and plasma metanephrines, elevated plasma cortisol levels despite dexamethasone suppression, elevated late-night salivary cortisol, and high-normal adrenocorticotrophic hormone. The patient underwent laproscopic right adrenalectomy, and pathology confirmed pheochromocytoma. Her lab abnormalities and symptoms of hot flashes and hypertension improved postoperatively. . This case demonstrates an unusual ACTH-secreting pheochromocytoma with subtle presentation and highlights the importance of obtaining a complete biochemical evaluation of incidental adrenal adenomas.
嗜铬细胞瘤是起源于交感肾上腺髓质嗜铬组织的罕见神经内分泌肿瘤。根据其分泌的儿茶酚胺量不同，临床表现各异。曾有分泌促肾上腺皮质激素（ACTH）的嗜铬细胞瘤导致严重库欣综合征的病例报道。在此，我们报告一例同时分泌促肾上腺皮质激素（ACTH）的嗜铬细胞瘤，因其罕见性和临床表现多样，可能是一个诊断难题。一名有结肠癌病史的64岁女性出现新发糖尿病、潮热加重和高血压。CT成像显示其右侧肾上腺结节增大（1.7厘米），衰减值为60亨氏单位，PET无摄取。生化评估显示尿和血浆间甲肾上腺素升高，尽管地塞米松抑制试验后血浆皮质醇水平仍升高，午夜唾液皮质醇升高，促肾上腺皮质激素处于高正常水平。该患者接受了腹腔镜右侧肾上腺切除术，病理证实为嗜铬细胞瘤。术后其实验室异常以及潮热和高血压症状均有所改善。本病例展示了一例表现隐匿的不寻常的分泌ACTH的嗜铬细胞瘤，并强调了对偶然发现的肾上腺腺瘤进行全面生化评估的重要性。
