Synonyms
Definition
ACTH-producing pancreatic tumour is a pancreatic neoplasm that synthetizes and secretes the adrenocorticotrophic hormone, causing the Cushing syndrome. This mostly includes functioning pancreatic neuroendocrine tumours (Pan-NETs) and this chapter will concentrate on these. Nevertheless, it is worth noting that rare acinar cell carcinomas (ACCs) may produce ACTH, with clinically evident Cushing syndrome, especially when arise in children.
Clinical Features
Incidence
Cushing syndrome related to ectopically produced ACTH accounts for 10% of the total. Of this, 4–16% of cases are due to a functioning, ACTH-producing, PanNET (Ilias et al. 2005). ACTH-producing PanNETs are very rare and less than 350 cases with pathological confirmation have been reported to date (Wu et al. 2021).
Age
Patients are affected at a younger age than other PanNETs, with more than two-thirds of patients being less than 50-year-old and a nonnegligible proportion of...
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References and Further Reading
Ilias, I., Torpy, D. J., Pacak, K., Mullen, N., Wesley, R. A., & Nieman, L. K. (2005). Cushing’s syndrome due to ectopic corticotropin secretion: Twenty years’ experience at the National Institutes of Health. The Journal of Clinical Endocrinology and Metabolism, 90, 4955–4962.
Maragliano, R., Vanoli, A., Albarello, L., et al. (2015). ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: Clinicopathologic study of 11 cases and review of the literature. The American Journal of Surgical Pathology, 39, 374–382.
Uccella, S., Maragliano, R., & Magnoli, F. (2015). ACTH-producing tumor. In S. La Rosa & F. Sessa (Eds.), Pancreatic neuroendocrine neoplasms (pp. 109–116). Cham: Springer.
Wu, Y., Xiong, G., Zhang, H., Wang, M., Zhu, F., & Qin, R. (2021). Adrenocorticotropic hormone-producing pancreatic neuroendocrine neoplasms: A systematic review. Endocrine Practice, 27, 152–157.
Young, J., Haissaguerre, M., Viera-Pinto, O., Chabre, O., Baudin, E., & Tabarin, A. (2020). Management of endocrine disease: Cushing’s syndrome due to ectopic ACTH secretion: An expert operational opinion. European Journal of Endocrinology, 182, R29–R58.
Zhang, C., Jin, J., Xie, J., et al. (2020). The clinical features and molecular mechanisms of acth-secreting pancreatic neuroendocrine tumors. The Journal of Clinical Endocrinology and Metabolism, 105, dgaa507.
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Uccella, S. (2022). ACTH-Producing Pancreatic Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5296-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5296-1
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