Murakami Keigo, Nakamura Yasuhiro, Felizola Saulo J A, Morimoto Ryo, Satoh Fumitoshi, Takanami Kentaro, Katakami Hideki, Hirota Seiichi, Takeda Yoshiyu, Meguro-Horike Makiko, Horike Shin-Ichi, Unno Michiaki, Sasano Hironobu
Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
Mol Cell Endocrinol. 2016 Nov 15;436:268-73. doi: 10.1016/j.mce.2016.08.044. Epub 2016 Aug 29.
Solitary fibrous tumors occasionally present with hypoglycemia because of the excessive release of insulin-like growth factor II. We report the first case of pancreatic solitary fibrous tumor causing ectopic adrenocorticotropic hormone syndrome. An 82-year-old Japanese man presented with lower limb edema, uncontrolled hypertension, hypokalemia, and baseline hypercortisolism. Distal pancreatectomy was performed after the clinical diagnosis of a neuroendocrine tumor with ectopic secretion of adrenocorticotropic hormone. On histological examination, the tumor showed spindle cells in a fascicular arrangement. The diagnosis of the solitary fibrous tumor was confirmed by the identification of the NAB2-STAT6 fusion gene and positive immuno-histochemical staining for STAT6 and CD34. Using quantitative real-time polymerase chain reaction, mRNA that encoded proopiomelanocortin, precursor of adrenocorticotropic hormone, was detected. Proopiomelanocortin production through the demethylation of the promoter region Domain IV was detected. Pancreatic solitary fibrous tumors represent a new cause of ectopic adrenocorticotropic hormone syndrome.
孤立性纤维瘤偶尔会因胰岛素样生长因子II的过度释放而出现低血糖症。我们报告首例导致异位促肾上腺皮质激素综合征的胰腺孤立性纤维瘤病例。一名82岁的日本男性出现下肢水肿、难以控制的高血压、低钾血症和基础皮质醇增多症。在临床诊断为异位分泌促肾上腺皮质激素的神经内分泌肿瘤后，进行了远端胰腺切除术。组织学检查显示肿瘤为束状排列的梭形细胞。通过鉴定NAB2-STAT6融合基因以及STAT6和CD34免疫组织化学染色阳性，确诊为孤立性纤维瘤。使用定量实时聚合酶链反应检测到编码促肾上腺皮质激素前体阿黑皮素原的mRNA。检测到通过启动子区域IV的去甲基化产生阿黑皮素原。胰腺孤立性纤维瘤是异位促肾上腺皮质激素综合征的一个新病因。
