Updates in Pancreatic Neuroendocrine Carcinoma

  • Susan Alsamarai Yale Cancer Center, Yale University School of Medicine. New Haven, CT, USA
  • Steven K Libutti Montefiore-Einstein Center for Cancer Care, Montefiore Medical Center, Albert Einstein College of Medicine. Bronx, NY, USA
  • Muhammad Wasif Saif Yale Cancer Center, Yale University School of Medicine. New Haven, CT, USA
Keywords: bevacizumab, mTOR protein, Neuroendocrine Tumors, sunitinib


Pancreatic neuroendocrine tumors display a range of clinical presentations and outcomes. Surgical resection remains the only potentially curative approach for primary tumors, and is also associated with a survival benefit for hepatic metastases as well. Data presented at the American Society of Clinical Oncology (ASCO) Annual Meeting this year suggest that targeted agents may also play a role in advanced disease. Sunitinib, which targets VEGF-1, 2 and 3 and PDGF seems to be a well tolerated treatment for advanced tumors. The mTOR inhibitor everolimus when combined with the VEGF inhibitor bevacizumab, resulted in measurable responses. The combination of bevacizumab and cytotoxic chemotherapy also shows potential.

Image: Smilow Cancer Hospital at Yale. New Haven, CT, USA.


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Halfdanarson TR, Rabe KG, Rubin J, Petersen GM. Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 2008; 19:1727-33. [PMID 18515795]

Key C. Cancer of the pancreas. In: Ries LAG, Young JL, Keel GE, Eisner MP, Lin YD, Horner M-J (editors). Chapter 7. SEER Survival Monograph: Cancer Survival Among Adults: U.S. SEER Program, 1988-2001, Patient and Tumor Characteristics. National Cancer Institute, SEER Program, NIH Pub. No. 07-6215, Bethesda, MD, 2007.

Ehehalt F, Saeger H, Schmidt C, Grützmann R. Neuroendocrine tumors of the pancreas. Oncologist 2009; 12:456-67. [PMID 19411317]

Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, Abdalla EK, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008; 26:3063-72. [PMID 18565894]

Pascher A, Klupp J, Neuhaus P. Endocrine tumours of the gastrointestinal tract. Transplantation in the management of metastatic endocrine tumours. Best Pract Res Clin Gastroenterol 2005; 19:637-648. [PMID 16183532]

Oberg K, Kvols L, Caplin M, Delle Fave G, de Herder W, Rindi G, et al. Consensus report on the use of somatostatin analogs for the management of neuroendocrine tumors of the gastroenteropancreatic system. Ann Oncol 2004; 15:966-73. [PMID 15151956]

Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, et al. 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; 27:4656-63. [PMID 19704057]

Moertel CG, Lefkopoulo M, Lipsitz S, Hah RG, Klaassen D. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 1992; 326:519-23. [PMID 1310159]

Eriksson B. New drugs in neuroendocrine tumors: rising of new therapeutic philosophies? Curr Opin Oncol 2010; 22:381-6. [PMID 20473165]

Moertel CG, Kvols LK, O'Connell MJ, Rubin J. Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer 1991; 68:227-32. [PMID 1712661]

Kulke MH, Hornick JL, Frauenhoffer C, Hooshmand S, Ryan DP, Enzinger PC, et al. O6-methylguanine DNA methyltransferase deficiency and response to temozolomide-based therapy in patients with neuroendocrine tumors. Clin Cancer Res 2009; 15:338-45. [PMID 19118063]

Kulke MH, Lenz HJ, Meropol NJ, Posey J, Ryan DP, Picus J, et al. Activity of sunitinib in patients with advanced neuroendocrine tumors. J Clin Oncol 2008; 26:3403-10. [PMID 18612155]

Yao JC, Phan AT, Chang DZ, Wolff RA, Hess K, Gupta S, et al. Efficacy of RAD001 (everolimus) and octreotide LAR in advanced low- to intermediate-grade neuroendocrine tumors: Results of a phase II trial. J Clin Oncol 2008; 26:4311-8. [PMID 18779618]

Yao JC, Lombard-Bohas C, Baudin E, Kvols LK, Rougier P, Ruszniewski P, et al. Daily oral everolimus activity in patients with metastatic pancreatic neuroendocrine tumors after failure of cytotoxic chemotherapy: a phase II trial. J Clin Oncol 2010; 28:69-76. [PMID 19933912]

Niccoli P, Raoul J, Bang Y, Borbath I, Lombard-Bohas C, Valle JW, et al. Updated safety and efficacy results of the phase III trial of sunitinib (SU) versus placebo (PBO) for treatment of pancreatic neuroendocrine tumors (NET). J Clin Oncol 2010; 28(15 Suppl):4000.

Raymond E, Niccoli P, Raoul J, Bang Y, Borbath I, Lombard-Bohas C, et al. Cox proportional hazard analysis of sunitinib (SU) efficacy across subgroups of patients (pts) with progressive pancreatic neuroendocrine tumors (NET). J Clin Oncol 2010; 28(15 Suppl):4031.

Vinik A, Bang Y, Raoul J, Valle JW, Metrakos P, Hörsch D, et al. Patient-reported outcomes (PROs) in patients (pts) with pancreatic neuroendocrine tumors (NET) receiving sunitinib (SU) in a phase III trial. J Clin Oncol 2010; 28(15 Suppl):4003.

Yao JC, Phan AT, Fogleman D, Ng CS, Jacobs CB, Dagohoy CD, et al. Randomized run-in study of bevacizumab (B) and everolimus (E) in low- to intermediate-grade neuroendocrine tumors (LGNETs) using perfusion CT as functional biomarker. J Clin Oncol 2010; 28(15 Suppl):4002.

Kunz PL, Kuo T, Zahn JM, Kaiser HL, Norton JA, Visser BC, et al. A phase II study of capecitabine, oxaliplatin, and bevacizumab for metastatic or unresectable neuroendocrine tumors. J Clin Oncol 2010; 28(15 Suppl):4104.

Smilow Cancer Hospital at Yale. New Haven, CT, USA
How to Cite
AlsamaraiS., LibuttiS., & SaifM. (2010). Updates in Pancreatic Neuroendocrine Carcinoma. JOP. Journal of the Pancreas, 11(4), 336-340. https://doi.org/10.6092/1590-8577/3618
Highlights from the “2010 ASCO Annual Meeting”. Chicago, IL, USA. June 4-8, 2010

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