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前列腺腺癌异位分泌促肾上腺皮质激素的疑难病例

Challenging Case of Ectopic ACTH Secretion from Prostate Adenocarcinoma.

作者信息

Zeng Wanling, Khoo Joan

机构信息

Department of Endocrinology, Changi General Hospital, 2 Simei Street 3 529889, Singapore.

出版信息

Case Rep Endocrinol. 2022 Mar 31;2022:3739957. doi: 10.1155/2022/3739957. eCollection 2022.

Abstract

Cushing's syndrome (CS) secondary to ectopic adrenocorticotrophic hormone (ACTH)-producing prostate cancer is rare with less than 50 cases reported. The diagnosis can be challenging due to atypical and variable clinical presentations of this uncommon source of ectopic ACTH secretion. We report a case of Cushing's syndrome secondary to prostate adenocarcinoma who presented with symptoms of severe hypercortisolism with recurrent hypokalaemia, limb oedema, limb weakness, and sepsis. He presented with severe hypokalaemia and metabolic alkalosis (potassium 2.5 mmol/L and bicarbonate 36 mmol/L), with elevated 8 am cortisol 1229 nmol/L. ACTH-dependent Cushing's syndrome was diagnosed with inappropriately normal ACTH 57.4 ng/L, significantly elevated 24-hour urine free cortisol and unsuppressed cortisol after 1 mg low-dose, 2-day low-dose, and 8 mg high-dose dexamethasone suppression tests. Ga-DOTANOC PET/CT showed an increase in DOTANOC avidity in the prostate gland, and his prostate biopsy specimen was stained positive for ACTH and markers for neuroendocrine differentiation. He was started on ketoconazole, which was switched to IV octreotide in view of liver dysfunction from hepatic metastases. He eventually succumbed to the disease after 3 months of his diagnosis. It is imperative to recognize prostate carcinoma as a source of ectopic ACTH secretion as it is associated with poor clinical outcomes, and the diagnosis can be missed due to atypical clinical presentations.

摘要

继发于分泌促肾上腺皮质激素(ACTH)的前列腺癌的库欣综合征(CS)较为罕见,报告病例不足50例。由于这种不常见的异位ACTH分泌来源的临床表现不典型且多变,诊断可能具有挑战性。我们报告一例继发于前列腺腺癌的库欣综合征病例,患者表现为严重皮质醇增多症的症状,伴有反复低钾血症、肢体水肿、肢体无力和脓毒症。他出现严重低钾血症和代谢性碱中毒(血钾2.5 mmol/L,碳酸氢根36 mmol/L),上午8点皮质醇升高至1229 nmol/L。通过促肾上腺皮质激素依赖的库欣综合征诊断,促肾上腺皮质激素水平异常正常为57.4 ng/L,24小时尿游离皮质醇显著升高,且在1 mg低剂量、2天低剂量及8 mg高剂量地塞米松抑制试验后皮质醇未被抑制。镓标记的奥曲肽PET/CT显示前列腺中奥曲肽摄取增加,其前列腺活检标本促肾上腺皮质激素及神经内分泌分化标志物染色呈阳性。他开始使用酮康唑治疗,鉴于肝转移导致肝功能障碍,改为静脉注射奥曲肽。诊断后3个月,他最终死于该疾病。必须认识到前列腺癌是异位ACTH分泌的一个来源,因为它与不良临床结局相关,且由于不典型的临床表现可能会漏诊。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0126/8989602/a7c39ab7f38a/CRIE2022-3739957.001.jpg

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