title: A case of ectopic adrenocorticotropic hormone-producing pancreatic neuroendocrine tumor with multiple liver metastases.
abstract: Ectopic adrenocorticotropic hormone (ACTH) production by the pancreatic neuroendocrine tumor (p-NET) is relatively rare, and patients with this tumor show poor prognosis. In this study, we present the case of a 64-year-old woman who presented with ectopic ACTH syndrome due to p-NET with multiple liver metastases. Computed tomography revealed that she had multiple masses in the liver and a solid mass in the head of the pancreas. Endocrinological examinations revealed markedly elevated plasma ACTH (735.0 pg/mL) and cortisol (34.7 microg/dL) levels associated with hypokalemia (2.7 mEq/L), diabetes and typical Cushingoid features. Histological examinations by needle biopsy of liver tumors in S5 and S8 indicated metastatic ACTH-producing NET, which was also confirmed by venous sampling. The metastatic live tumor was somatostatin receptor (SSTR)-2a- and SSTR-5-positive as revealed by immunohistochemical staining, and reverse transcription polymerase chain reaction revealed divergent expression patterns of SSTRs, pro-opiomelanocortin, and gastrin mRNA. To avoid complications of hypercortisolemia, metyrapone was first administered to reduce the cortisol levels. After near-normalization of cortisol levels, transarterial chemoembolization and somatostatin analogue treatment were performed. The combination of these treatments effectively decreased ACTH and cortisol levels and also ameliorated hyperglycemia. We have achieved controlled hormone secretion and prevented tumor growth in this patient for more than 20 months, suggesting that highly individualized treatment for NET should be undertaken because of its divergent and heterogeneous characteristics.
authors.affiliation: Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Kondo
authors.forename: Rina
authors.initials: R
authors.lastname: Matsuyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ashihara
authors.forename: Yasuto
authors.initials: Y
authors.lastname: Matsuo
authors.forename: Kazunari
authors.initials: K
authors.lastname: Sasaki
authors.forename: Rieko
authors.initials: R
authors.lastname: Goto
authors.forename: Kaoru
authors.initials: K
authors.lastname: Ono
authors.forename: Yuki
authors.initials: Y
authors.lastname: Takaki
authors.forename: Yumi
authors.initials: Y
authors.lastname: Honda
authors.forename: Ken-Ichi
authors.initials: K
authors.lastname: Iyama
authors.forename: Junji
authors.initials: J
authors.lastname: Kawashima
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Motoshima
authors.forename: Kaku
authors.initials: K
authors.lastname: Tsuruzoe
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Miyamura
authors.forename: Eiichi
authors.initials: E
authors.lastname: Araki
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood*; D016461:Chemoembolization, Therapeutic; D005260:Female; D006729:Hormones, Ectopic / Q000097:blood*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D033162:Incidental Findings; D008113:Liver Neoplasms / Q000556:secondary* / Q000628:therapy; D008797:Metyrapone / Q000627:therapeutic use; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000473:pathology*; D015282:Octreotide / Q000627:therapeutic use; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000628:therapy; D017481:Receptors, Somatostatin / Q000378:metabolism; D014057:Tomography, X-Ray Computed
chemical_list: D006729:Hormones, Ectopic; D017481:Receptors, Somatostatin; C094573:somatostatin receptor 5; D000324:Adrenocorticotropic Hormone; C093733:somatostatin receptor 2; D015282:Octreotide; D006854:Hydrocortisone; D008797:Metyrapone