Gottschalk J, Buntrock P, Gerl H, Knappe G, Rohde W
Zentralbl Allg Pathol. 1985;130(5):369-74.
Ectopic production of ACTH is observed in 6% of patients with Cushing syndrome. Ten percent of these cases are related to endocrine pancreatic tumors. In a few cases a multiplicity of hormones are produced. The combination with a Zollinger-Ellison syndrome is very infrequent. In the present case, a female patient aged 54, there was an interval between the onset of Zollinger-Ellison and Cushing syndrome. The combination of bilaterally enlarged adrenals in the absence of an adrenal adenoma and the presence of Crooke-cells in the adenohypophysis in a patient with Cushing syndrome are of diagnostic significance. This trias should always alert the physician to the possibility of extrahypophyseal ACTH production.
在6%的库欣综合征患者中观察到促肾上腺皮质激素(ACTH)异位分泌。其中10%的病例与内分泌胰腺肿瘤有关。在少数病例中会产生多种激素。与佐林格-埃利森综合征合并的情况非常罕见。在本病例中,一名54岁的女性患者,佐林格-埃利森综合征和库欣综合征发病之间有一段间隔时间。库欣综合征患者双侧肾上腺增大但无肾上腺腺瘤,且腺垂体存在克罗克细胞,这种组合具有诊断意义。这种三联征应始终提醒医生注意垂体外ACTH分泌的可能性。