Ectopic ACTH-Secreting Syndrome: A Retrospective Study Over 11 Years From a Single Center

Chen-Yi Liao ； Kuang-Yu Wei ； Ming-Feng Tseng ； Chia-Chao Wu ； Sung-Sen Yang

《Acta Nephrologica》 32卷4期 (2018/12) Pp. 151-159

https://doi.org/10.6221/AN.201812_32(4).0002

摘要

BACKGROUND: Ectopic adrenocorticotropic hormone (ACTH)-secreting syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze the clinical and biochemical characteristics, tumor location, management strategies, and outcomes of EAS patients. METHODS: We screened the records (2006-2017) of cases diagnosed with Cushing's syndrome (CS) that were managed at a tertiary care center according to the International Classification of Diseases 9th edition codes. RESULTS: Of the 95 patients, 6 were diagnosed with EAS, 6 were diagnosed with Cushing's disease, 13 were diagnosed with adrenal adenoma and the remaining 76 were diagnosed with iatrogenic CS. In the EAS group, the mean 8:00 AM serum cortisol level was 55.80 μg/dL (4.8-19.5 μg/dL), and the mean basal plasma ACTH level was 162.9 pg/mL (0.1-46.0 pg/mL). Notably, ACTH levels ＜ 100 pg/mL were found in two patients. The mean potassium level was 2.5 mmol/L. Computed tomography localized the lesion in 6/6 patients. The cure rate was borderline (50%). CONCLUSION: In EAS patients, ACTH-secreting tumors that originated in the chest cavity were the primary cause of the condition and may be underestimated in some cases because of atypical presentation. Hypokalemia with metabolic alkalosis may be an early symptom. The EAS group had the lowest potassium levels and the highest infection rate among patients with CS. Furthermore, the mean diastolic pressure was higher in the adrenal adenoma group than in the other groups. We therefore suggest careful evaluation of patients with neuroendocrine tumors should avoid missing co-existing EAS.

關鍵字

Cushing's disease ； Cushing's syndrome ； ectopic ACTH-secreting syndrome ； hypokalemia ； metabolic akalosis