A Case of Ectopic Adrenocorticotropic Hormone-producing Pancreatic Neuroendocrine Tumor with Multiple Liver Metastases

Tatsuya KONDO; Rina MATSUYAMA; Hiroshi ASHIHARA; Yasuto MATSUO; Kazunari SASAKI; Rieko GOTO; Kaoru ONO; Yuki TAKAKI; Yumi HONDA; Ken-ichi IYAMA; Junji KAWASHIMA; Hiroyuki MOTOSHIMA; Kaku TSURUZOE; Nobuhiro MIYAMURA; Eiichi ARAKI

doi:10.1507/endocrj.K09E-179

ORIGINALS

A Case of Ectopic Adrenocorticotropic Hormone-producing Pancreatic Neuroendocrine Tumor with Multiple Liver Metastases

Tatsuya KONDO, Rina MATSUYAMA, Hiroshi ASHIHARA, Yasuto MATSUO, Kazunari SASAKI ^†, Rieko GOTO ^†, Kaoru ONO, Yuki TAKAKI, Yumi HONDA, Ken-ichi IYAMA, Junji KAWASHIMA, Hiroyuki MOTOSHIMA, Kaku TSURUZOE, Nobuhiro MIYAMURA, Eiichi ARAKI

Author information

Tatsuya KONDO
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Rina MATSUYAMA
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Hiroshi ASHIHARA
Department of Gastroenterology and Hepatology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Yasuto MATSUO
Department of Diabetology, Saiseikai Kumamoto Hospital, Kumamoto, Japan
Kazunari SASAKI ^†
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Rieko GOTO ^†
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Kaoru ONO
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Yuki TAKAKI
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Yumi HONDA
Department of Surgical Pathology, Kumamoto University, Kumamoto, Japan
Ken-ichi IYAMA
Department of Surgical Pathology, Kumamoto University, Kumamoto, Japan
Junji KAWASHIMA
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Hiroyuki MOTOSHIMA
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Kaku TSURUZOE
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Nobuhiro MIYAMURA
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Eiichi ARAKI
Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
Author contributions

†These authors contributed equally: Kazunari SASAKI, Rieko GOTO

Keywords: ACTH, Cortisol, Ectopic Cushing’s syndrome, Octreotide, Somatostatin receptors

JOURNAL FREE ACCESS

2010 Volume 57 Issue 3 Pages 229-236

DOI https://doi.org/10.1507/endocrj.K09E-179

View "Advance Publication" version (December 22, 2009).

Details

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 μg/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.

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