Definition
Calcitonin-producing pancreatic tumor is a pancreatic neuroendocrine neoplasm (PanNEN) that synthetize and secrete calcitonin. This mostly includes pancreatic neuroendocrine tumor (Pan-NET), but also pancreatic neuroendocrine carcinoma (PanNEC) may be calcitonin-producing. Calcitonin-producing PanNENs are associated with high circulating levels of calcitonin and, clinically, must be differentiated from medullary thyroid carcinoma. Notably, calcitonin-producing PanNETs may also produce other biologically active hormones (e.g., insulin, somatostatin, VIP, and others), being associated with the related clinical syndrome.
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References and Further Reading
Ansari, U. A., Ramirez, C. L., Feliberti, E. C., Vinik, A. I., & Perry, R. R. (2017). Calcitonin-secreting pancreatic neuroendocrine tumor in a patient with multiple endocrine neoplasia Type 1. AACE Clinical Case Reports, 3, 317–321.
Jeong, Y. J., Oh, H. K., & Bong, J. G. (2014). Multiple endocrine neoplasia type 1 associated with breast cancer: A case report and review of the literature. Oncology Letters, 8, 230–234.
Nozières, C., Chardon, L., Goichot, B., Borson-Chazot, F., Hervieu, V., Chikh, K., Lombard-Bohas, C., & Walter, T. (2016). Neuroendocrine tumors producing calcitonin: Characteristics, prognosis and potential interest of calcitonin monitoring during follow-up. European Journal of Endocrinology, 174, 335–341.
Schneider, R., Waldmann, J., Swaid, Z., Ramaswamy, A., Fendrich, V., Bartsch, D. K., & Schlosser, K. (2011). Calcitonin-secreting pancreatic endocrine tumors: Systematic analysis of a rare tumor entity. Pancreas, 40, 213–221.
Uccella, S., Blank, A., Maragliano, R., Sessa, F., Perren, A., & La Rosa, S. (2017). Calcitonin-producing neuroendocrine neoplasms of the pancreas: Clinicopathological study of 25 cases and review of the literature. Endocrine Pathology, 28, 351–361.
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Uccella, S. (2022). Calcitonin-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_5300-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5300-1
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