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异位 ACTH 综合征通常发生于起源神经嵴的肿瘤,包括肺、胰腺、胸腺、内分泌腺、交感神经系统和副神经节组织(paraganglionic tissue)的肿瘤。发生于鼻窦的嗜铬细胞肿瘤产生异位 ACTH 的柯兴氏综合征尚未见报告,作者报告1例。女性,50岁。因严重衰弱、右侧鼻部堵塞、注意力不集中、记忆力不好和多尿症住院。检查 BP140/100,典型的类柯兴氏综合征表现,右侧鼻孔有黄色息肉样的肿块堵塞。血钠147mEq/L,血糖470mg/dl,尿素23mg/dl,钙9.2mg/dl,血色素13.9g。ECG 显示 U 波。头颅和鼻窦 X 线片显示肿物累及右侧筛窦。蝶窦和上颌窦,并破坏了上颌窦侧壁和眼眶
Ectopic ACTH syndrome usually occurs in tumors of neural crest origin, including lung, pancreas, thymus, endocrine glands, sympathetic nervous system, and paraganglionic tissue tumors. The Cushing’s Syndrome in which ectopic ACTH occurs in paraffin-embedded chromosomal neoplasms has not yet been reported, and the authors report 1 case. Female, 50 years old. Severe debilitation, right nasal blockage, poor concentration, poor memory, and polyuria. Examination of BP140/100, typical Cushing’s syndrome, showed a yellow polyp-like mass obstruction in the right nostril. Blood sodium 147mEq/L, blood glucose 470mg/dl, urea 23mg/dl, calcium 9.2mg/dl, hemoglobin 13.9g. ECG shows U wave. Head and sinus radiographs show that the tumor involving the right ethmoid sinus. Sphenoid sinus and maxillary sinus, and destroy the maxillary sinus lateral wall and orbit