Tadokoro Rie, Sato Shotaro, Otsuka Fumiko, Ueno Makoto, Ohkawa Shinichi, Katakami Hideki, Taniyama Matsuo, Nagasaka Shoichiro
Department of Medicine, Division of Diabetes, Metabolism and Endocrinology, Showa University Fujigaoka Hospital, Japan.
Intern Med. 2016;55(20):2979-2983. doi: 10.2169/internalmedicine.55.6827. Epub 2016 Oct 15.
The patient was a 61-year-old woman who had a well-differentiated pancreatic neuroendocrine tumor (PNET) with lymph node metastasis. After 15 months of octreotide treatment, glucose control deteriorated and pigmentation of the tongue and moon face developed, leading to the diagnosis of ectopic adrenocorticotropic hormone (ACTH) syndrome. An abnormal secretion of growth hormone (GH) was identified, and the plasma growth hormone-releasing hormone (GHRH) level was elevated. A tumor biopsy specimen positively immunostained for ACTH and GHRH. Ectopic hormone secretion seems to have evolved along with the progression of the PNET.
该患者为一名61岁女性，患有伴有淋巴结转移的高分化胰腺神经内分泌肿瘤（PNET）。在接受15个月的奥曲肽治疗后，血糖控制恶化，出现舌部色素沉着和满月脸，从而诊断为异位促肾上腺皮质激素（ACTH）综合征。发现生长激素（GH）分泌异常，血浆生长激素释放激素（GHRH）水平升高。肿瘤活检标本对ACTH和GHRH免疫染色呈阳性。异位激素分泌似乎随着PNET的进展而演变。
