Cellular classifications of pancreatic neuroendocrine tumors (islet cell tumors) | Lairmore, T. C., Quinn, C. E., & Martinez, M. J. (2013). Neuroendocrine tumors of the pancreas: molecular pathogenesis and current surgical management. Translational Gastrointestinal Cancer; Vol 3, No 1 (January 2014): Translational Gastrointestinal Cancer. Retrieved from http://tgc.amegroups.com/article/view/2774Nomenclature, incidence, location and malignancy of pancreatic NETs | Lairmore, T. C., Quinn, C. E., & Martinez, M. J. (2013). Neuroendocrine tumors of the pancreas: molecular pathogenesis and current surgical management. Translational Gastrointestinal Cancer; Vol 3, No 1 (January 2014): Translational Gastrointestinal Cancer. Retrieved from http://tgc.amegroups.com/article/view/2774
Very rare: Pancreatic polypeptide cells & enterochromaffin cells
Aetiology
Genetic mutations:
Germline mutations in the menin tumor suppressor gene may represent missense, nonsense, deletion, or RNA splicing defects and are distributed anywhere along the 9 coding exons and the intron-exon junctions of the gene. | Mutch MG et al. Human mutation 1999;13:175-85 (53).
MEN1 mutation → Multiple endocrine neoplasia (MEN) Type 1 (tumours in parathyroid, pituitary & pancreas)
Common Genes: PNET vs Pancreatic Adenocarcinoma (PDAC) | Enets. (2019) Pancreatic Neuroendocrine Tumors: State-of-the-Art Diagnosis and Management: Page 3 of 3 | Cancer Network. Retrieved December 30, 2019, from https://www.cancernetwork.com/neuroendocrine-tumors/pancreatic-neuroendocrine-tumors-state-art-diagnosis-and-management/page/0/2
Common functional pNETs and their syndromes | Reynolds, R. B., & Folloder, J. (2014). Clinical Management of Pancreatic Cancer. Journal of the Advanced Practitioner in Oncology, 5(5), 356–364. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/26114016
Diagnosis
WHO histologic grading (2010):
2010 WHO grading system for pNETs
TNM staging:
Management
Treatment Algorithm. Suggested treatment algorithm for pancreatic neuroendocrine tumors, based on functionality and localized or metastatic disease. | Authors Links. (2019) Cancers | Free Full-Text | Treatment Options for Pancreatic Neuroendocrine Tumors | HTML. Retrieved December 30, 2019, from https://www.mdpi.com/2072-6694/11/6/828/htm#
Summary of all surgical options available to deal with PanNETs. A tailored, single-patient, focused approach remains the best option. | Lairmore, T. C., Quinn, C. E., & Martinez, M. J. (2013). Neuroendocrine tumors of the pancreas: molecular pathogenesis and current surgical management. Translational Gastrointestinal Cancer; Vol 3, No 1 (January 2014): Translational Gastrointestinal Cancer. Retrieved from http://tgc.amegroups.com/article/view/2774
Management of pancreatic neuroendocrine tumors: enucleation vs. pancreatic resection. Depicted on the left is enucleation of a small, apparently benign solitary insulinoma of the pancreatic tail. On the right is shown a pancreaticoduodenectomy specimen for a large malignant NET of the head of the pancreas. | Lairmore, T. C., Quinn, C. E., & Martinez, M. J. (2013). Neuroendocrine tumors of the pancreas: molecular pathogenesis and current surgical management. Translational Gastrointestinal Cancer; Vol 3, No 1 (January 2014): Translational Gastrointestinal Cancer. Retrieved from http://tgc.amegroups.com/article/view/2774