Pancreatic solitary fibrous tumor causing ectopic adrenocorticotropic hormone syndrome

Keigo Murakami, Yasuhiro Nakamura, Saulo J.A. Felizola, Ryo Morimoto, Fumitoshi Satoh, Kentaro Takanami, Hideki Katakami, Seiichi Hirota, Yoshiyu Takeda, Makiko Meguro-Horike, Shin ichi Horike, Michiaki Unno, Hironobu Sasano

研究成果: Article査読

5 被引用数 (Scopus)


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.

ジャーナルMolecular and Cellular Endocrinology
出版ステータスPublished - 2016 11 15

ASJC Scopus subject areas

  • 生化学
  • 分子生物学
  • 内分泌学


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