Neuroendocrine Tumors: Treatment Updates

  • Simon Khagi Tufts Medical Center, Tufts University School of Medicine. Boston, MA, USA
  • Muhammad Wasif Saif Tufts Medical Center, Tufts University School of Medicine. Boston, MA, USA
Keywords: bevacizumab, Carcinoid Tumor, everolimus, Neuroendocrine Tumors, Octreotide, pasireotide, Somatostatin, temsirolimus


Neuroendocrine tumors of the gastroenteropancreatic tract remain a difficult array of neoplasia to treat. Treatment of advanced and metastatic gastroenteropancreatic neuroendocrine tumors has traditionally been difficult with few systemic treatment options. In 2011, two new targeted therapies, everolimus and sunitinib were approved for treatment of pancreatic neuroendocrine tumor. The approval of these agents led to an enhanced interest in exploring novel agents. This can be evidenced by the fact that this is the first year that ASCO assembled related abstracts under a separate title of neuroendocrine tumor. The annual American Society of Clinical Oncology (ASCO) conference in 2013 presented four abstracts (#4030, #4031, #4032, #4136) that shed light on new therapeutic options that help target the unique pathways involved in these neuroendocrine malignancies.

Image: Nuclear octreotide scintigraphy.


Download data is not yet available.


Oberg K, Funa K, Alm G. Effects of leukocyte interferon on clinical symptoms and hormone levels in patients with mid-gut carcinoid tumors and carcinoid syndrome. N Engl J Med. 1983;309(3):129. PMID 6191217

Granberg D, Eriksson B, Wilander E, Grimfjärd P, Fjällskog ML, Oberg K, Skogseid B. Experience in treatment of metastatic pulmonary carcinoid tumors. Ann Oncol. 2001;12(10):1383. PMID 11762808

Rubin J, Ajani J, Schirmer W, Venook AP, Bukowski R, Pommier R, Saltz L, Dandona P, Anthony L. Octreotide acetate long-acting formulation versus open-label subcutaneous octreotide acetate in malignant carcinoid syndrome. J Clin Oncol. 1999;17(2):600. PMID 10080605

Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R, PROMID Study Group. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol. 2009;27(28):4656. PMID 19704057

Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D. Streptozocin-doxorubicin, streptozocin-fluorouracil or chloro-zotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med. 1992;326(8):519. PMID 1310159

Ramanathan RK, Cnaan A, Hahn RG, Carbone PP, Haller DG. Phase II trial of dacarbazine (DTIC) in advanced pancreatic islet cell carcinoma. Study of the Eastern Cooperative Oncology Group-E6282. Ann Oncol. 2001;12(8):1139. PMID 11583197

Kulke MH, Stuart K, Enzinger PC, Ryan DP, Clark JW, Muzikansky A, Vincitore M, Michelini A, Fuchs CS. Phase II study of temozolomide and thalidomide in patients with metastatic neuroendocrine. J Clin Oncol. 2006;24(3):401. PMID 16421420

Strosberg JR, Fine RL, Choi J, Nasir A, Coppola D, Chen DT, Helm J, Kvols L. First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer. 2011;117(2):268. PMID 20824724

Missiaglia E, Dalai I, Barbi S, Beghelli S, Falconi M, della Peruta M, Piemonti L, Capurso G, Di Florio A, delle Fave G, Pederzoli P, Croce CM, Scarpa A. Pancreatic endocrine tumors: expression profiling evidences a role for AKT-mTOR pathway. J Clin Oncol. 2010;28(2):245. PMID 19917848

Kulke MH, Lenz HJ, Meropol NJ, Posey J, Ryan DP, Picus J, Bergsland E, Stuart K, Tye L, Huang X, Li JZ, Baum CM, Fuchs CS. Activity of sunitinib in patients with advanced neuroendocrine tumors. J Clin Oncol. 2008;26(20):3403. PMID 18612155

Yao JC, Shah MH, Ito T, Bohas CL, Wolin EM, Van Cutsem E, Hobday TJ, Okusaka T, Capdevila J, de Vries EG, Tomassetti P, Pavel ME, Hoosen S, Haas T, Lincy J, Lebwohl D,Öberg K, RAD001 in Advanced Neuroendocrine Tumors, Third Trial (RADIANT-3) Study Group. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011;364(6):514. PMID 21306238

Pavel ME, Hainsworth JD, Baudin E, Peeters M, Hörsch D, Winkler RE, Klimovsky J, Lebwohl D, Jehl V, Wolin EM, Oberg K, Van Cutsem E, Yao JC, RADIANT-2 Study Group. Everolimus plus octreotide long-acting repeatable for the treatment of advanced neuroendocrine tumours associated with carcinoid syndrome (RADIANT-2): a randomised, placebo-controlled, phase 3 study. Lancet. 2011;378(9808):2005. PMID 22119496

Hobday TJ, Qin R, Reidy DL, et al. Multicenter phase II trial of temsirolimus and bevacizumab in pancreatic neuroendocrine tumor (abstract). J Clin Oncol 2012; 30 (suppl 4): Abstract 260.

Yao JC, Phan A, Hoff PM, Chen HX, Charnsangavej C, Yeung SC, Hess K, Ng C, Abbruzzese JL, Ajani JA. Targeting vascular endothelial growth factor in advanced carcinoid tumor: a random assignment phase II study of depot octreotide with bevacizumab and pegylated interferon alpha-2b. J Clin Oncol. 2008;26(8):1316. PMID 18323556

R. Arnold, M. Wittenberg, A. Rinke, C. Schade-Brittinger, B. Aminossadati, E. Ronicke, T. M. Gress, H. H. Mueller, PROMID Study Group. Placebo controlled, double blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors (PROMID): Results on long-term survival. ASCO 2013 Abstract No: #4030

E. M. Wolin, B. Jarzab, B. Eriksson, T. Walter, C. Toumpanakis, M. Morse, P. Tomassetti, M. Weber, D. R. Fogelman, J. Ramage, D. Poon, J. M. Huang, M. Hudson, X. Zhi, J. L. Pasieka, A. Mahamat, F. Swahn, J. Newell-Price, W. Mansoor, K. E. Oberg. A multicenter, randomized, blinded, phase III study of pasireotide LAR versus octreotide LAR in patients with metastatic neuroendocrine tumors (NET) with disease-related symptoms inadequately controlled by somatostatin analogs. ASCO 2013 Abstract No:#4031

E. Bajetta, L. Catena, N. Fazio, S. Pusceddu, P. Biondani, G. Blanco, S. Ricci, M. Aieta, F. Pucci, M. Valente, N. Bianco, F. Bellomo, C. Mauri, P. Buonandi, V. Roberto Everolimus in combination with octreotide LAR as the first-line treatment for advanced neuroendocrine tumors: A phase II trial of the I.T.M.O. (Italian Trials in Medical Oncology) group. ASCO 2013 Abstract No: #4136

T. J. Hobday, R. Qin, M. J. Moore, D. L. Reidy, J. R. Strosberg, H. L. Kindler, M. H. Shah, H. Lenz, A. Kaubisch, H. X. Chen, C. Erlichman. Multicenter phase II trial of temsirolimus (TEM) and bevacizumab (BEV) in pancreatic neuroendocrine tumor (PNET). ASCO 2013 Abstract No: #4032

Nuclear octreotide scintigraphy
How to Cite
KhagiS., & SaifM. (2013). Neuroendocrine Tumors: Treatment Updates. JOP. Journal of the Pancreas, 14(4), 367-371.
Highlights from the “2013 ASCO Annual Meeting”. Chicago, IL, USA. May 31 - June 4, 2013