A case of ectopic adrenocorticotropic hormone-producing pancreatic neuroendocrine tumor with multiple liver metastases.
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.
Kondo
Tatsuya
T
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
Matsuyama
Rina
R
Ashihara
Hiroshi
H
Matsuo
Yasuto
Y
Sasaki
Kazunari
K
Goto
Rieko
R
Ono
Kaoru
K
Takaki
Yuki
Y
Honda
Yumi
Y
Iyama
Ken-Ichi
K
Kawashima
Junji
J
Motoshima
Hiroyuki
H
Tsuruzoe
Kaku
K
Miyamura
Nobuhiro
N
Araki
Eiichi
E
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
D006729:Hormones, Ectopic; D017481:Receptors, Somatostatin; C094573:somatostatin receptor 5; D000324:Adrenocorticotropic Hormone; C093733:somatostatin receptor 2; D015282:Octreotide; D006854:Hydrocortisone; D008797:Metyrapone