Neuroendocrine tumours (NETs) represent a wide spectrum of disease. They consist of a large heterogeneous group of malignancies that are derived from embryonic neural crest tissue found in various organ such as the hypothalamus, pituitary gland, thyroid gland, adrenal medulla, and gastrointestinal tract.
The spectrum of neuroendocrine tumours range from:
- carcinoid tumour: arising from enterochromaffin cells and can occur at various sites such as thymus, lung, ovary
- phaeochromocytoma: typically occurs in the adrenals but can also occur in extra-adrenal sites
- location dependant entities
They can also be divided by their biochemical behaviour as functioning or non-functioning, by their aggressiveness as slow-growing and more aggressive lesions and so on.
Because of secretory products by amine precursor uptake and decarboxylation (APUD) they were historically known as APUDomas.
Mostly occur sporadically, some are linked to multiple endocrine neoplasia syndromes.
- 1. Speel EJ, Richter J, Moch H et-al. Genetic differences in endocrine pancreatic tumor subtypes detected by comparative genomic hybridization. Am. J. Pathol. 1999;155 (6): 1787-94. doi:10.1016/S0002-9440(10)65495-8 - Free text at pubmed - Pubmed citation
- 2. Rindi G, Leiter AB, Kopin AS et-al. The "normal" endocrine cell of the gut: changing concepts and new evidences. Ann. N. Y. Acad. Sci. 2004;1014: 1-12. Pubmed citation
- 3. Rindi G, Wiedenmann B. Neuroendocrine neoplasms of the gut and pancreas: new insights. Nat Rev Endocrinol. 2011;8 (1): 54-64. doi:10.1038/nrendo.2011.120 - Pubmed citation
- 4. Kulke MH, Mayer RJ. Carcinoid tumors. N. Engl. J. Med. 1999;340 (11): 858-68. doi:10.1056/NEJM199903183401107 - Pubmed citation
- 5. Reznek RH. CT/MRI of neuroendocrine tumours. Cancer Imaging. 2006;6 (Special Issue A): S163-77. doi:10.1102/1470-7330.2006.9037 - Free text at pubmed - Pubmed citation
- 6. Intenzo CM, Jabbour S, Lin HC et-al. Scintigraphic imaging of body neuroendocrine tumors. Radiographics. 2007;27 (5): 1355-69. doi:10.1148/rg.275065729 - Pubmed citation
- 7. Raman SP, Hruban RH, Cameron JL et-al. Pancreatic imaging mimics: part 2, pancreatic neuroendocrine tumors and their mimics. AJR Am J Roentgenol. 2012;199 (2): 309-18. doi:10.2214/AJR.12.8627 - Pubmed citation