NDLI: Cushing's Syndrome Due to CRH and ACTH Co-secreting Pancreatic Tumor--Presentation of a New Case Focusing on Diagnostic Pitfalls.

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Cushing's Syndrome Due to CRH and ACTH Co-secreting Pancreatic Tumor--Presentation of a New Case Focusing on Diagnostic Pitfalls.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Fountas, Athanasios Giotaki, Zoe Ligkros, Nikos Tsakiridou, Efthymia D. Tigas, Stelios Saeger, Wolfgang Tsatsoulis, Agathocles
Description	Author Affiliation: Fountas A ( Department of Endocrinology, University Hospital of Ioannina, Stavros Niarchos Avenue, 45110, Ioannina, Greece, thanasisfountas@gmail.com.)
Abstract	Cushing's syndrome (CS) due to a corticotropin-releasing hormone (CRH) and adrenocorticotropin hormone (ACTH) co-secreting tumor is very rare, and diagnosis can be difficult. We describe a case of CS caused by ectopic CRH and ACTH production from a pancreatic neuroendocrine tumor (pNET) and discuss possible pitfalls in the diagnosis. A 48-year-old woman presented with gradual increase in body weight, muscle weakness, and difficult to control hypertension. Laboratory and imaging investigations revealed ACTH-dependent hypercortisolemia and a 3-cm mass at the head of the pancreas. The patient underwent partial pancreatoduodenectomy. Histological examination revealed a well-differentiated pNET, expressing both CRH and ACTH. After a follow-up period of 18 months, she remains asymptomatic with no metastatic disease. This is the fourth case report of CS in adults due to an ectopic ACTH- and CRH-secreting pNET. Co-secretion of ACTH and CRH by the same tumor may cause diagnostic problems since investigation depends on which of the two hormones is secreted in greater amounts. Measurement of plasma CRH may help in establishing the diagnosis, especially in patients in whom endocrine tests are indicative of ectopic CS, whereas imaging findings are suggestive of pituitary hyperplasia.
File Format	HTM / HTML
ISSN	10463976
Issue Number	3
Volume Number	26
e-ISSN	15590097
Journal	Endocrine Pathology
Language	English
Publisher	Springer
Publisher Date	2015-09-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Endocrinology Discipline Pathology Acth Syndrome, Ectopic Diagnosis Adrenocorticotropic Hormone Secretion Corticotropin-releasing Hormone Cushing Syndrome Neuroendocrine Tumors Pancreatic Neoplasms Pathology Metabolism Diagnosis, Differential Female Humans Middle Aged Case Reports Journal Article
Content Type	Text
Resource Type	Case study Article
Subject	Endocrinology, Diabetes and Metabolism Pathology and Forensic Medicine Endocrinology

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