嗜铬细胞瘤或副神经节瘤(PPGL)起源于嗜铬细胞可以产生多种激素,除了儿茶酚胺,包括促肾上腺皮质激素(ACTH)。在嗜铬细胞瘤中,高水平的ACTH可能不会导致色素沉着,通常在艾迪生病中观察到,患者可能不会表现出库欣综合征的症状,尽管ACTH依赖性皮质醇增多症。一名63岁的男性高血压患者入院,计算机断层扫描(CT)显示右肾上腺大肿瘤。尽管血浆ACTH(700-1300pg/mL)和血清皮质醇(90-100µg/dL)水平高,未观察到身体色素沉着或库欣果症状。尿中去甲肾上腺素和去甲肾上腺素水平高达16.0mg和3.2mg,分别。123I-间碘苄基胍(MIBG)闪烁显像阴性。低剂量地塞米松矛盾地增加ACTH和皮质醇水平,表明糖皮质激素对两种激素的潜在正反馈调节。该患者被诊断出患有产生ACTH的嗜铬细胞瘤,并在静脉内给予大剂量α-阻断剂和氢化可的松的情况下进行了成功的腹腔镜手术以切除肾上腺肿瘤。ACTH的水平,皮质醇,肿瘤切除后,尿中的间肾上腺素/去甲肾上腺素恢复正常。我们报告了一例罕见的嗜铬细胞瘤,其ACTH/皮质醇产生极高,但没有色素沉着或库欣果症状。我们还回顾了以前关于糖皮质激素对ACTH/皮质醇的矛盾调节的产生ACTH的PPGL的报道,色素沉着,Cushingoid症状,和123I-MIBG闪烁显像的负性。
Pheochromocytoma or paraganglioma (PPGL) originating from chromaffin cells can produce diverse hormones in addition to catecholamines, including adrenocorticotropic hormone (ACTH). In pheochromocytoma, high levels of ACTH might not result in
pigmentation as typically observed in Addison\'s disease, and patients might not exhibit the symptoms of Cushing\'s syndrome, despite ACTH-dependent hypercortisolism. A 63-year-old male patient with hypertension was admitted to our facility, and computed tomography (CT) revealed a large right adrenal tumor. Despite high plasma ACTH (700-1300 pg/mL) and serum cortisol (90-100 µg/dL) levels, no physical
pigmentation or Cushingoid symptoms were observed. Urinary metanephrine and normetanephrine levels reached as high as 16.0 mg and 3.2 mg, respectively. 123I-metaiodobenzylguanidine (MIBG) scintigraphy was negative. Low-dose dexamethasone paradoxically increased ACTH and cortisol levels, indicating the potential positive feedback regulation of both hormones by glucocorticoids. The patient was diagnosed with an ACTH-producing pheochromocytoma and underwent successful laparoscopic surgery to remove the adrenal tumor under the intravenous administration of a high-dose α-blocker and hydrocortisone. The levels of ACTH, cortisol, and urinary metanephrine/normetanephrine returned close to normal after tumor removal. We report a rare
case of pheochromocytoma with extremely high ACTH/cortisol production but without
pigmentation or Cushingoid symptoms. We also reviewed previous
reports of ACTH-producing PPGL regarding the paradoxical regulation of ACTH/cortisol by glucocorticoids,
pigmentation, Cushingoid symptoms, and negativity of 123I-MIBG scintigraphy.