Nakahira Hironori, Miyauchi Shozo, Watanabe Kyoko, Akesaka Kazuyuki, Ono Keizo, Ebisui Osamu, Miyake Teruki, Furukawa Shinya, Hiasa Yoichi, Matsuura Bunzo
Clinical Training Center, Ehime Prefectural Central Hospital, Ehime 790-0024, Japan.
Department of Diabetes and Endocrinology, Ehime Prefectural Central Hospital, Ehime 790-0024, Japan.
Endocr J. 2025 Jun 2;72(6):743-750. doi: 10.1507/endocrj.EJ24-0459. Epub 2025 Feb 14.
Pheochromocytomas occur in the adrenal medulla and present with various symptoms associated with excessive catecholamine production. Although pheochromocytomas associated with ectopic ACTH-producing tumors and hyper-interleukin-6emia (hyper-IL-6emia) have been reported, those associated with both diseases simultaneously have not been reported. In pheochromocytomas with ectopic ACTH-producing tumors and hyper-IL-6emia, the disease characteristics and relationship between each hormone and cytokine are unknown. Herein, we report a case of a 56-year-old woman with stroke whose computed tomography scans of the abdomen revealed a right adrenal tumor on systemic examination. Endocrinological examination revealed elevated plasma levels of catecholamines and their metabolites in the urine and elevated levels of plasma ACTH, serum cortisol, and serum dehydroepiandrosterone sulfate, leading to a diagnosis of right pheochromocytoma and associated ectopic ACTH-producing tumor. Furthermore, hyper-IL-6emia was detected as a key indicator of anemia due to inflammatory hematopoietic disorders. The patient's general condition improved with drug therapy, including 1,000 mg/d of metyrapone, 2 mg/h of phentolamine, 8 mg/d of doxazosin, and systemic management. Dexamethasone suppression tests demonstrated suppressed serum cortisol and IL-6 levels, and dexamethasone dose-dependently increased plasma adrenaline and noradrenaline levels. These findings indicate that excess glucocorticoids play a stimulatory role in catecholamine secretion and a concentration-suppressive role in serum IL-6 levels in pheochromocytomas associated with ectopic ACTH-producing tumors and hyper-IL-6emia. The presence of rare comorbidities should be considered if the clinical findings cannot be explained by the pathophysiology of a pheochromocytoma alone because pheochromocytomas can be associated with the production of other hormones and cytokines.
嗜铬细胞瘤发生于肾上腺髓质，表现为与儿茶酚胺分泌过多相关的各种症状。虽然已有与异位促肾上腺皮质激素（ACTH）分泌肿瘤和高白细胞介素-6血症（高IL-6血症）相关的嗜铬细胞瘤的报道，但同时与这两种疾病相关的病例尚未见报道。在伴有异位ACTH分泌肿瘤和高IL-6血症的嗜铬细胞瘤中，疾病特征以及每种激素与细胞因子之间的关系尚不清楚。在此，我们报告一例56岁的中风女性患者，其腹部计算机断层扫描在全身检查时发现右肾上腺肿瘤。内分泌检查显示血浆儿茶酚胺及其尿代谢产物水平升高，血浆ACTH、血清皮质醇和血清硫酸脱氢表雄酮水平升高，从而诊断为右嗜铬细胞瘤及相关的异位ACTH分泌肿瘤。此外，检测到高IL-6血症是炎症性造血障碍所致贫血的关键指标。患者的一般状况通过药物治疗得到改善，包括每天1000毫克甲吡酮、每小时2毫克酚妥拉明、每天8毫克多沙唑嗪以及全身管理。地塞米松抑制试验显示血清皮质醇和IL-6水平受到抑制，且地塞米松剂量依赖性地增加血浆肾上腺素和去甲肾上腺素水平。这些发现表明，在伴有异位ACTH分泌肿瘤和高IL-6血症的嗜铬细胞瘤中，过量的糖皮质激素对儿茶酚胺分泌起刺激作用，对血清IL-6水平起浓度抑制作用。如果临床发现不能仅由嗜铬细胞瘤的病理生理学来解释，就应考虑罕见合并症的存在，因为嗜铬细胞瘤可能与其他激素和细胞因子的产生有关。
