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Licensed Unlicensed Requires Authentication Published by De Gruyter November 12, 2020

Ectopic ACTH production by thymic and appendiceal neuroendocrine tumors – two case reports

Elżbieta Moszczyńska ORCID logo, Katarzyna Pasternak-Pietrzak ORCID logo, Monika Prokop-Piotrkowska, Anna Śliwińska, Sylwia Szymańska and Mieczysław Szalecki



Ectopic adrenocorticotropic syndrome (EAS) causes approximately 10–18% of cases of Cushing’s syndrome (CS) in adults, while in children it occurs much less frequently.

Case presentation

We report two cases of neuroendocrine tumors (of the thymus and the appendix) in a 12-year-old boy and a 15-year-old girl who presented with the clinical features of CS. Elevated serum cortisol, ACTH, and chromogranin levels were observed in both patients. Diagnoses were made on the basis of a mass in the thymus/appendix region visualized with chest/abdominal CT scan and radiotracer accumulation in scintigraphy in the same areas. Histopathological examinations confirmed the diagnoses of NET.


EAS is an extremely rare endocrine disorder. However, it should be taken into consideration in the diagnostic process of every case of ACTH-dependent CS.

Corresponding author: Katarzyna Pasternak-Pietrzak, MD, Department of Endocrinology and Diabetology, The Children’s Memorial Health Institute, Al. Dzieci Polskich 20,04-730Warsaw, Poland, Phone: +48228157578, E-mail:

  1. Research funding: None declared.

  2. Author contributions: All the authors have accepted the responsibility for the entire content of this submitted manuscript and approved the submission.

  3. Competing interest: The authors declare no conflicts of interest.

  4. Informed consent: Informed consent was obtained from all individuals included in this study.

  5. Ethical approval: We hereby state that the subjects and their parents gave their written informed consent to publish their cases.


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Received: 2020-07-23
Accepted: 2020-09-28
Published Online: 2020-11-12
Published in Print: 2021-01-27

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