Neuroendocrine neoplasms or NENs are on the rise; typically found in the GI tract, pancreas, lungs and liver; here we summarise emerging treatments for these rare cancers.
Neuroendocrine cancer, also called a Neuroendocrine tumour or NET, is a cancer that begins in neuroendocrine cells - commonly in cells of the endocrine (hormone) system or occasionally in cells of the nervous system. Strictly speaking, the majority are endocrine cancers. They most often occur in the gastrointestinal tract (small intestine, rectum, colon), where they are called carcinoid tumours, and are also found in the pancreas and lung and, more rarely, other locations such as the liver. Because these rare cancers can arise from the endocrine cells, they may also produce hormones.
Although many originally were described as benign, they are now all classified as malignant, but some are very slow growing. One major issue is that the early tumours cause hormonal issues which may build over a period of five to seven years before the patient is diagnosed with a cancer. Other symptoms can vary from bowel obstruction to diarrhoea, uncontrolled blood sugar and gastric ulcers.
Typical treatment is surgery and chemotherapy with drugs such as Temozolomide (1) and/or Capecitabine (2, 3). Immunotherapy is also being explored with NETs (4) and particularly the simultaneous use of Ipilimumab and Nivolumab (5), although there have been reports of liver problems in CANCERactive patients. Targeted therapies such as Everolimus and Sunitinib are also useful according to the American Cancer Society.
A novel approach from MD Anderson uses the hormone receptors on the tumour. They use the particular hormone to drag radiation to the tumour killing more of the NET and saving more of the surrounding healthy cells. This is called Peptide Receptor Radionuclide Therapy, or PRRT (6). It was approved by the FDA in 2018 and reduces tumours by 80%.
Go to: Pancreatic cancer overview
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References
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Temozolomide as monotherapy is effective in treatment of advanced malignant neuroendocrine tumors; Clin Canc Res 2007, Mat 15; 13(10) Sara Ekeblad et al - https://pubmed.ncbi.nlm.nih.gov/17505000/#affiliation-1
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Capecitabine and temozolomide (CAPTEM) for metastatic, well-differentiated neuroendocrine cancers: The Pancreas Center at Columbia University experience; 2013; March 7. - https://pubmed.ncbi.nlm.nih.gov/23370660/
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Outcomes of Capecitabine and Temozolomide (CAPTEM) in Advanced Neuroendocrine Neoplasms (NENs); MDPI, 14 January 2020; https://www.mdpi.com/2072-6694/12/1/206/htm
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"Present and future of immunotherapy in Neuroendocrine Tumors”; Rev Endocr Metab Disord 2021 Sep;22; - https://pubmed.ncbi.nlm.nih.gov/33851319/
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Immunotherapy of Ipilimumab and Nivolumab in Patients with Advanced Neuroendocrine Tumors; Clin Canc Res; 2020 Sep 1;26(17); Oliver Klein et al; https://pubmed.ncbi.nlm.nih.gov/32532787/
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Neuroendocrine tumours at MD Anderson - 9 things to know: https://www.mdanderson.org/cancerwise/neuroendocrine-tumors--9-things-to-know.h00-159379578.html