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

Research output: Contribution to journalArticlepeer-review

7 Citations (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.

Original languageEnglish
Pages (from-to)268-273
Number of pages6
JournalMolecular and Cellular Endocrinology
Publication statusPublished - 2016 Nov 15


  • Adrenocorticotropic hormone
  • Ectopic adrenocorticotropic hormone syndrome
  • Pancreas
  • Proopiomelanocortin
  • Solitary fibrous tumour


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