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Pituitary

, Volume 22, Issue 5, pp 561–568 | Cite as

Ectopic cushing’s syndrome due to corticotropin releasing hormone

  • Manouchehr Nakhjavani
  • Alireza Amirbaigloo
  • Soghra Rabizadeh
  • Fabio Rotondo
  • Kalman Kovacs
  • Ali A. GhaziEmail author
Article
  • 165 Downloads

Abstract

Cushing’s syndrome (CS) secondary to corticotropin releasing hormone (CRH) producing tumors is rare. In this paper we present an Iranian patient who was admitted to our hospital with classic signs and symptoms of CS. Laboratory evaluation revealed high serum and urine cortisol which could not be suppressed with dexamethasone. Abdominal CT scan revealed a mass in abdominal cavity. A percutaneous needle biopsy was performed and histopathologic evaluation revealed that the mass was a neuroendocrine tumor. A multi-disciplinary approach including resection of the mass, bilateral adrenalectomy somatostatin analogue and chemotherapy was applied for management of the disease. Extensive review of English literature focusing on the topic from 1971 to 2018 revealed that there have been only 75 similar cases. Clinical, laboratory, imaging, histopathologic characteristics and managements of these patients will also be discussed in this paper.

Keywords

Cushing’s syndrome Ectopic Corticotropin-releasing hormone producing tumor CRH producing tumor 

Notes

Acknowledgement

Authors are grateful to the Jarislowsky and Lloyd Carr-Harris foundations for their support.

Compliance with ethical standards

Conflict of interest

Authors have no conflicts of interest to declare.

Ethical approval

This work has been conducted with the consent of the patient and in accordance to the standards of institutional/national Ethics Committee. All work also conforms to the provisions of the Declaration of Helsinki and Tokyo.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Manouchehr Nakhjavani
    • 1
  • Alireza Amirbaigloo
    • 2
  • Soghra Rabizadeh
    • 1
  • Fabio Rotondo
    • 3
    • 4
  • Kalman Kovacs
    • 3
    • 4
  • Ali A. Ghazi
    • 5
    Email author
  1. 1.Endocrinology and Metabolism Research Center (EMRC), Vali-Asr Hospital, Tehran University of Medical SciencesTehranIran
  2. 2.Endocrinologist, Practicing in Private OfficeKarajIran
  3. 3.Department of Laboratory Medicine, Division of PathologyTorontoCanada
  4. 4.The Keenan Research Centre for Biomedical Science at the Li Ka Shing Knowledge Institute, St. Michael’s Hospital, University of TorontoTorontoCanada
  5. 5.Endocrine Research Center, Research Institute for Endocrine Sciences (RIES), Shahid Beheshti University of Medical SciencesTehranIran

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