title: Pancreatic solitary fibrous tumor causing ectopic adrenocorticotropic hormone syndrome.
abstract: 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.
authors: Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan. Electronic address: kehgo@med.tohoku.ac.jp.
authors: Keigo
authors: K
authors: Murakami
authors: Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Yasuhiro
authors: Y
authors: Nakamura
authors: Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Saulo J A
authors: SJ
authors: Felizola
authors: Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Ryo
authors: R
authors: Morimoto
authors: Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Fumitoshi
authors: F
authors: Satoh
authors: Department of Radiology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Kentaro
authors: K
authors: Takanami
authors: Clinical Research Science, Department of Medicine, Teikyo University Chiba Medical Center, Chiba, 299-0111, Japan.
authors: Hideki
authors: H
authors: Katakami
authors: Department of Surgical Pathology, Hyogo College of Medicine, Hyogo, 663-8501, Japan.
authors: Seiichi
authors: S
authors: Hirota
authors: Department of Internal Medicine, Kanazawa University Graduate School of Medical Science, Kanazawa, 920-0934, Japan.
authors: Yoshiyu
authors: Y
authors: Takeda
authors: Advanced Science Research Center, Institute for Gene Research, Kanazawa University, Kanazawa, 920-0934, Japan.
authors: Makiko
authors: M
authors: Meguro-Horike
authors: Advanced Science Research Center, Institute for Gene Research, Kanazawa University, Kanazawa, 920-0934, Japan.
authors: Shin-Ichi
authors: S
authors: Horike
authors: Department of Surgery, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Michiaki
authors: M
authors: Unno
authors: Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8574, Japan.
authors: Hironobu
authors: H
authors: Sasano
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001483:Base Sequence; D019175:DNA Methylation / Q000235:genetics; D018076:DNA, Complementary / Q000235:genetics; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D054364:Solitary Fibrous Tumors / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D013577:Syndrome
chemical_list: D018076:DNA, Complementary; D000324:Adrenocorticotropic Hormone
keywords: Adrenocorticotropic hormone; Ectopic adrenocorticotropic hormone syndrome; Pancreas; Proopiomelanocortin; Solitary fibrous tumour