Definition
Insulinoma is a well-differentiated functioning neuroendocrine tumor associated with an uncontrolled secretion of insulin by tumor cells, causing a hypoglycaemic syndrome.
Clinical Features
Incidence
Insulinomas have an estimated annual incidence of 0.4 cases per 100,000 person and represent the most common functioning pancreatic neuroendocrine tumor (Service et al. 1991).
Age
Insulinomas can affect any age, but they are rare in children and young adults with an incidence <1% in patients <30 year. The pick of incidence is observed in the sixth decade (Mehrabi et al. 2014).
Sex
Females seem to be affected slightly more frequently than males.
Site
Most insulinomas are located in the pancreas, although rare duodenal cases have been described as well (La Rosa et al. 2013).
Treatment
Surgery in the treatment of choice and, since most of cases are small, enucleation is the surgical approach generally performed. In...
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References and Further Reading
Cao, Y., Gao, Z., Li, L., et al. (2013). Whole exome sequencing of insulinoma reveals recurrent T372R mutations in YY1. Nature Communications, 4, 2810.
La Rosa, S., Pariani, D., Calandra, C., Marando, A., Sessa, F., Cortese, F., & Capella, C. (2013). Ectopic duodenal insulinoma: A very rare and challenging tumor type. Description of a case and review of the literature. Endocrine Pathology, 24, 213–219.
Marinoni, I., Kurrer, A. S., Vassella, E., Dettmer, M., Rudolph, T., Banz, V., Hunger, F., Pasquinelli, S., Speel, E. J., & Perren, A. (2014). Loss of DAXX and ATRX are associated with chromosome instability and reduced survival of patients with pancreatic neuroendocrine tumors. Gastroenterology, 146, 453–460.
Mehrabi, A., Fischer, L., Hafezi, M., et al. (2014). A systematic review of localization, surgical treatment options, and outcome of insulinoma. Pancreas, 43, 675–686.
Missiaglia, E., Dalai, I., et al. (2010). Pancreatic endocrine tumors: Expression profiling evidences a role for AKT-mTOR pathway. Journal of Clinical Oncology, 28, 245–255.
Rindi, G., Falconi, M., Klersy, C., et al. (2012). TNM staging of neoplasms of the endocrine pancreas: Results from a large international cohort study. Journal of the National Cancer Institute, 104, 764–777.
Sadanandam, A., Wullschleger, S., Lyssiotis, C. A., et al. (2015). A cross-species analysis in pancreatic neuroendocrine tumors reveals molecular subtypes with distinctive clinical, metastatic, developmental, and metabolic characteristics. Cancer Discovery, 5, 1296–1313.
Service, F. J., McMahon, M. M., O’Brien, P. C., & Ballard, D. J. (1991). Functioning insulinoma--incidence, recurrence, and long-term survival of patients: A 60-year study. Mayo Clinic Proceedings, 66, 711–719.
Siddiqui, M., Vora, A., Ali, S., Abramowitz, J., & Mirfakhraee, S. (2020). Pasireotide: A novel treatment for tumor-induced hypoglycemia due to insulinoma and non-islet cell tumor hypoglycemia. J Endocr Soc, 5, bvaa171.
Wang, L., Yang, M., Zhang, Y., Xu, S., & Tian, B. L. (2015). Prognostic validation of the WHO 2010 grading system in pancreatic insulinoma patients. Neoplasma, 62, 484–490.
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La Rosa, S. (2021). Insulinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5190-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5190-1
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