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Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing's syndrome
- Publication Year :
- 2016
- Publisher :
- D.A. Spandidos, 2016.
-
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.
- Subjects :
- Cancer Research
Pathology
medicine.medical_specialty
endocrine system
Adrenocorticotropic hormone
Neuroendocrine tumors
digestive system
03 medical and health sciences
0302 clinical medicine
Biopsy
medicine
030212 general & internal medicine
Ampulla
biology
medicine.diagnostic_test
business.industry
Ampulla of Vater
Chromogranin A
Combination chemotherapy
Articles
medicine.disease
digestive system diseases
medicine.anatomical_structure
Oncology
030220 oncology & carcinogenesis
Synaptophysin
biology.protein
business
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....70595427517c1fbd066f66c875e5e6d4