Definition
Appendiceal neuroendocrine neoplasms (NEN) are neoplasms belonging to the neuroendocrine category, that is, neoplasms composed by tumor cells expressing markers of neuroendocrine differentiation. Appendiceal NENs include well-differentiated neuroendocrine tumors (NET), poorly differentiated neuroendocrine carcinomas (NECs), and mixed neuroendocrine-non neuroendocrine neoplasms (MiNEN) (WHO Classification of Tumours Editorial Board. 2019). NET and NEC are characterized by the expression of neuroendocrine markers in all tumor cells. MiNEN are characterized by the expression of neuroendocrine markers in at least 30% of tumor cells.
References and Further Reading
Borazanci, E., Millis, S. Z., Kimbrough, J., Doll, N., Von Hoff, D., & Ramanathan, R. K. (2017). Potential actionable targets in appendiceal cancer detected by immunohistochemistry, fluorescent in situ hybridization, and mutational analysis. Journal of Gastrointestinal Oncology, 8(1), 164–172. https://doi.org/10.21037/jgo.2017.01.14.
Dasari, A., Shen, C., Halperin, D., Zhao, B., Zhou, S., Xu, Y., et al. (2017). Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncology, 3(10), 1335–1342. https://doi.org/10.1001/jamaoncol.2017.0589.
Leoncini, E., Boffetta, P., Shafir, M., Aleksovska, K., Boccia, S., & Rindi, G. (2017). Increased incidence trend of low-grade and high-grade neuroendocrine neoplasms. Endocrine, 58(2), 368–379. https://doi.org/10.1007/s12020-017-1273-x.
WHO Classification of Tumours Editorial Board. (2019). Digestive system tumours (Vol. 1, 5th ed.). Lyon: International Agency for Research on Cancer.
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Rindi, G., Inzani, F. (2020). Appendiceal Neuroendocrine Neoplasms. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5262-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5262-1
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