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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

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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.

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Funding

This research did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sector.

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The authors declare that they have no competing interests.

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Authors and Affiliations

  1. Department of Endocrinology, University Hospital of Ioannina, Stavros Niarchos Avenue, 45110, Ioannina, Greece

    Athanasios Fountas, Zoe Giotaki, Nikos Ligkros, Efthymia D. Tsakiridou, Stelios Tigas & Agathocles Tsatsoulis

  2. Institute of Neuropathology, University of Hamburg, Hamburg, Germany

    Wolfgang Saeger

Authors
  1. Athanasios Fountas
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  2. Zoe Giotaki
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  3. Nikos Ligkros
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  4. Efthymia D. Tsakiridou
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  5. Stelios Tigas
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  6. Wolfgang Saeger
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  7. Agathocles Tsatsoulis
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Correspondence to Agathocles Tsatsoulis.

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Fountas, A., Giotaki, Z., Ligkros, N. et al. Cushing’s Syndrome Due to CRH and ACTH Co-secreting Pancreatic Tumor—Presentation of a New Case Focusing on Diagnostic Pitfalls. Endocr Pathol 26, 239–242 (2015). https://doi.org/10.1007/s12022-015-9384-5

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  • DOI: https://doi.org/10.1007/s12022-015-9384-5

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