Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing's syndrome

Kato,Akihisa; Hayashi,Kazuki; Naitoh,Itaru; Seno,Kyoji; Okada,Yukiko; Ban,Tesshin; Kondo,Hiromu; Nishi,Yuji; Umemura,Shuichiro; Hori,Yasuki; Natsume,Makoto; Joh,Takashi

doi:10.3892/mco.2016.869

July-2016 Volume 5 Issue 1

Full Size Image

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

An International Open Access Journal Devoted to General Medicine.

July-2016 Volume 5 Issue 1

Full Size Image

Article

Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing's syndrome

Authors:
- Akihisa Kato
- Kazuki Hayashi
- Itaru Naitoh
- Kyoji Seno
- Yukiko Okada
- Tesshin Ban
- Hiromu Kondo
- Yuji Nishi
- Shuichiro Umemura
- Yasuki Hori
- Makoto Natsume
- Takashi Joh
View Affiliations / Copyright

Affiliations: Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya 467‑8601, Japan, Department of Gastroenterology, Nagoya West Medical Center, Nagoya 462-8508, Japan, Department of Internal Medicine, Kasugai Municipal Hospital, Kasugai 486-8510, Japan
Pages: 113-116
|
Published online on: April 21, 2016

https://doi.org/10.3892/mco.2016.869
Expand metrics +

Abstract

Ectopic adrenocorticotropic hormone (ACTH) is rarely secreted by neuroendocrine tumors. Although neuroendocrine tumors may occur at any site in the gastrointestinal system, they very rarely occur in the ampulla of Vater and have a poor prognosis. The present study described the first Cushing's syndrome as a result of ectopic ACTH arising from the ampulla of Vater neuroendocrine carcinoma. A 69‑year‑old female was admitted with clinical features of Cushing's syndrome, confirmed biochemically by hypokalemia, and elevated levels of ACTH and cortisol. In further investigations, a tumor of the ampulla of Vater and liver metastases were detected. Pathological analysis of the biopsy confirmed a neuroendocrine carcinoma, which was immunohistochemically positive for chromogranin A, synaptophysin, cluster of differentiation 56 and ACTH. Therefore, the present study diagnosed a functional and metastatic neuroendocrine carcinoma of the ampulla of Vater with ectopic ACTH production causing Cushing's syndrome. The patient succumbed to mortality 4 months later, despite administration of combined chemotherapy with irinotecan and cisplatin.

View Figures

View References

1	Selvakumar E, Rajendran S, Balachandar TG, Kannan DG, Jeswanth S, Ravichandran P and Surendran R: Neuroendocrine carcinoma of the ampulla of Vater: A clinicopathologic evaluation. Hepatobiliary Pancreat Dis Int. 7:422–425. 2008.PubMed/NCBI
2	Ricci JL: Carcinoid of the ampulla of Vater. Local resection or pancreaticoduodenectomy. Cancer. 71:686–690. 1993. View Article : Google Scholar : PubMed/NCBI
3	Riggs BL Jr and Sprague RG: Association of Cushing's syndrome and neoplastic disease: Observations in 232 cases of Cushing's syndrome and review of the literature. Arch Intern Med. 108:841–849. 1961. View Article : Google Scholar : PubMed/NCBI
4	Jex RK, van Heerden JA, Carpenter PC and Grant CS: Ectopic ACTH syndrome. Diagnostic and therapeutic aspects. Am J Surg. 149:276–282. 1985. View Article : Google Scholar : PubMed/NCBI
5	Oberg K: Neuroendocrine tumors (NETs): Historical overview and epidemiology. Tumori. 96:797–801. 2010.PubMed/NCBI
6	Rindi G, Petrone G and Inzani F: The 2010 WHO classification of digestive neuroendocrine neoplasms: A critical appraisal four years after its introduction. Endocr Pathol. 25:186–192. 2014. View Article : Google Scholar : PubMed/NCBI
7	Albores-Saavedra J, Hart A, Chablé-Montero F and Henson DE: Carcinoids and high-grade neuroendocrine carcinomas of the ampulla of vater: A comparative analysis of 139 cases from the surveillance, epidemiology, and end results program-a population based study. Arch Pathol Lab Med. 134:1692–1696. 2010.PubMed/NCBI
8	Solcia E, Fiocca R, Rindi G, Villani L, Luinetti O, Burrell M, Bosi F and Silini E: Endocrine tumors of the small and large intestine. Pathol Res Pract. 191:366–372. 1995. View Article : Google Scholar : PubMed/NCBI
9	Wajchenberg BL, Mendonca BB, Liberman B, Pereira MA, Carneiro PC, Wakamatsu A and Kirschner MA: Ectopic adrenocorticotropic hormone syndrome. Endocr Rev. 15:752–787. 1994. View Article : Google Scholar : PubMed/NCBI
10	Stewart PM, Walker BR, Holder G, O'Halloran D and Shackleton CH: 11 beta-Hydroxysteroid dehydrogenase activity in Cushing's syndrome: Explaining the mineralocorticoid excess state of the ectopic adrenocorticotropin syndrome. J Clin Endocrinol Metab. 80:3617–3620. 1995. View Article : Google Scholar : PubMed/NCBI
11	Arteaga E, Fardella C, Campusano C, Cárdenas I and Martinez P: Persistent hypokalemia after successful adrenalectomy in a patient with Cushing's syndrome due to ectopic ACTH secretion: Possible role of 11beta-hydroxysteroid dehydrogenase inhibition. J Endocrinol Invest. 22:857–859. 1999. View Article : Google Scholar : PubMed/NCBI
12	Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, Hu MI, Waguespack SG, Jimenez C, Ying AK, Cabanillas M, Abbara M and Habra MA: Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: The university of texas MD anderson cancer center experience. Cancer. 117:4381–4389. 2011. View Article : Google Scholar : PubMed/NCBI
13	Klimstra DS, Modlin IR, Coppola D, Lloyd RV and Suster S: The pathologic classification of neuroendocrine tumors: A review of nomenclature, grading, and staging systems. Pancreas. 39:707–712. 2010. View Article : Google Scholar : PubMed/NCBI
14	Jayant M, Punia R, Kaushik R, Sharma R, Sachdev A, Nadkarni NK and Attri A: Neuroendocrine tumors of the ampulla of vater: Presentation, pathology and prognosis. JOP. 13:263–267. 2012.PubMed/NCBI
15	Odabasi M, Yildiz KM, Cengiz E, Hasan AH, Gunay E, Ozkan E, Aktekin A, Kaya B and Muftuoglu TM: Treatment of ampullary neuroendocrine tumor by endoscopic snare papillectomy. Am J Case Rep. 14:439–443. 2013. View Article : Google Scholar : PubMed/NCBI
16	Yamaguchi T, Machida N, Morizane C, Kasuga A, Takahashi H, Sudo K, Nishina T, Tobimatsu K, Ishido K, Furuse J, et al: Multicenter retrospective analysis of systemic chemotherapy for advanced neuroendocrine carcinoma of the digestive system. Cancer Sci. 105:1176–1181. 2014. View Article : Google Scholar : PubMed/NCBI

Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing's syndrome

This article is mentioned in:

Abstract

Figure 1

Figure 2

Related Articles