Gerl H, Knappe G, Rohde W, Stahl F, Wolff H, Martin H
Klinik für Innere Medizin Theodor Brugsch, Humboldt-Universität, Berlin.
Dtsch Med Wochenschr. 1990 Mar 2;115(9):332-6. doi: 10.1055/s-2008-1065011.
A 36-year-old patient developed marked pigmentation, marked myopathy and severe hypokalaemic alkalosis which at first pointed towards an ectopic ACTH syndrome. The dexamethasone test at a high dose indicated cortisol suppression. A mediastinal tumour was seen radiologically, but the sella was of normal size. Computed tomography provided indirect signs of a sellar space-occupying lesion which suggested an ectopic production of corticotropin-releasing factor (CRF) as cause of the Cushing's syndrome. CRF concentration in antecubital venous blood was markedly elevated to 280 ng/l. The mediastinal tumour was excised and proved to be a carcinoid histologically. Postoperatively the CRF concentration fell to 70 ng/l. An extract of the carcinoid contained 15.5 ng/g wet-weight of CRF and 254 ng/g wet-weight of beta-endorphin. The patient died 5 weeks postoperatively of sepsis with bilateral pneumonia. At autopsy the hypophysis was of normal size but showed nodular ACTH-cell hyperplasia. This was thus a case of Cushing's syndrome resulting from ectopic CRF production in a mediastinal carcinoid tumour.
一名36岁患者出现明显色素沉着、明显肌病和严重低钾性碱中毒，起初提示为异位促肾上腺皮质激素（ACTH）综合征。大剂量地塞米松试验显示皮质醇受到抑制。影像学检查发现纵隔肿瘤，但蝶鞍大小正常。计算机断层扫描提供了蝶鞍占位性病变的间接征象，提示促肾上腺皮质激素释放因子（CRF）异位产生是库欣综合征的病因。肘前静脉血中CRF浓度显著升高至280 ng/l。切除纵隔肿瘤，组织学检查证实为类癌。术后CRF浓度降至70 ng/l。类癌提取物中CRF含量为15.5 ng/g湿重，β-内啡肽含量为254 ng/g湿重。患者术后5周死于败血症合并双侧肺炎。尸检时垂体大小正常，但显示结节性促肾上腺皮质激素细胞增生。因此，这是一例由纵隔类癌肿瘤异位产生CRF导致的库欣综合征病例。
