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What are neuroendocrine tumors?

Neuroendocrine tumors (NETs) are a rare form of cancer with just 5-6 new cases per 100,000 individuals diagnosed each year.1 NETs originate in neuroendocrine cells and often develop slowly over several years. Healthy neuroendocrine cells are part of both the endocrine and the nervous system and can be found throughout the body. These cells release neurotransmitters, cell messengers or hormones into the blood, which control how the body works, meaning they play a key role in the interaction between the nervous and endocrine system.

75 % of all neuroendocrine tumors are from gastroenteric or pancreatic origin and are referred to as gastroenteric pancreatic neuroendocrine tumors (GEPNETs).2 GEP-NETs are found throughout the digestive system or related parts of the body. in the digestive system. GEP-NETs are often asymptomatic and challenging to diagnose. This is why they are often diagnosed at a late stage with metastases and limited possibility of surgery. GEP-NETs appear predominantly in patients aged 50-60 with women being approximately 2.5 times more often affected than men.3 In some cases GEP-NETs can secrete various hormones and are then referred to as functioning GEP-NETs.

Healthy neuroendocrine cells have somatostatin receptors on their surface. The neurotransmitter somatostatin regulates the endocrine system within normal body function by binding to these receptors according to the lock and key principle. Tumor cells of neuroendocrine origin often express an increased number of these somatostatin receptors on their surface, showing a unique tumor characteristic that differs from that of healthy neuroendocrine cells.4

Neuroendocrine tumors are often detected via PET/CT (Positron Emission Tomography / Computerized Tomography) or SPECT (Single Photon Emission Computed Tomography) diagnostic imaging techniques. Surgery is the main treatment for NETs and often the only course of treatment
needed. However, NETs can spread to other parts of the body, or your clinician may not be able to completely remove the cancer by surgery alone. If this happens, other treatment options such as chemotherapy or targeted drugs are applied.

One targeted therapy for the treatment of NETs in the pancreas and small bowel, Everolimus, is currently defined as one of the standard of care treatments. Other treatment options for NETs, including Targeted Radionuclide Therapy with n.c.a. Lutetium-177-Edotreotide, are being investigated in clinical trials to improve patient care.

 

1 Bryan Oronsky et al., December 2017, Neoplasia Vol. 19, pp. 991-1002
2 Pape et al., 2000, Der Onkologe 6: 624–633
3 Bryan Oronsky et al., December 2017, Neoplasia Vol. 19, pp. 991-1002
4 Papotti et al., 2002, Virchows Archiv 440(5): 461-75