Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medical Sciences, Japan.
Department of Diabetes, Endocrinology and Metabolism, Koseiren Takaoka Hospital, Japan.
Intern Med. 2022 Dec 15;61(24):3693-3698. doi: 10.2169/internalmedicine.9238-21. Epub 2022 May 31.
Localization of ectopic cyclic Cushing's syndrome, which causes life-threatening complications, is challenging. A 70-year-old woman showed cyclic hypokalemia and hyperglycemia and was diagnosed with cyclic ectopic Cushing's syndrome. Although somatostatin-receptor scintigraphy failed to localize the responsible tumor, fluorodeoxyglucose-positron emission tomography (FDG-PET) showed the uptake of tracer in a lung tumor. Lobectomy resulted in remission. The resected adrenocorticotropic hormone (ACTH)-producing neuroendocrine tumor had Ki-67<2% and negative staining for somatostatin receptors. This is the first case assessed both radiological findings and pathological findings in cyclic ectopic Cushing's syndrome. Subsequent FDG-PET is recommended if somatostatin-receptor scintigraphy is negative.
异位库欣综合征的定位具有挑战性,因为它会引起危及生命的并发症。一位 70 岁女性表现为周期性低钾血症和高血糖,被诊断为周期性异位库欣综合征。尽管生长抑素受体闪烁显像未能定位责任肿瘤,但氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)显示示踪剂在肺肿瘤中的摄取。肺叶切除术缓解了病情。切除的促肾上腺皮质激素(ACTH)产生的神经内分泌肿瘤 Ki-67<2%,且生长抑素受体染色阴性。这是首例评估周期性异位库欣综合征的影像学和病理学发现的病例。如果生长抑素受体闪烁显像为阴性,建议进行后续 FDG-PET 检查。