Abstract
Pancreatic neuroendocrine neoplasms (PNENs) are most commonly advanced, low/intermediate-grade of malignancy, nonfunctioning, and somatostatin receptor expressing. However in a small percentage of cases, they are high-grade of malignancy, and prognosis is poor independently from therapy. Medical therapy of PNENs is usually considered in advanced stage of disease. It should be based on the right diagnosis, prognostic estimate, and morphological and functional characterization of disease. Several different options can be considered, including chemotherapy, somatostatin analogs, interferon, molecular-targeted agents, peptide receptor radionuclide therapy, and liver-directed treatments. While in high-grade PNENs, chemotherapy represents the only therapeutic option; in low-/intermediate-grade PNENs, several options should be discussed within a multidisciplinary team. No studies defining sequence, timing, and integration of the different therapies exist so far; therefore, a specific therapeutic strategy for the single patient should be discussed and shared by means of an interactive interdisciplinary discussion, preferably involving a referral center for NEN.
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References
Jensen RT, Berna MJ, Bingham DB, Norton JA (2008) Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer 113:1807–1843
Bosman (2010) WHO classification of tumor of the digestive system. IARC Press, Lyon
Moertel CG, Kvols LK, O’Connel MJ (1991) Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Cancer 68:227–232
Mitry E, Baudin E, Ducreaux M et al (1999) Treatment of poorly differentiated neuroendocrine tumours with etoposide and cisplatin. Br J Cancer 81:1351–1355
Fjallskog MLH, Granberg DPH, Welin SLW et al (2001) Treatment with cisplatin and etoposide in patients with neuroendocrine tumors. Cancer 92(5):1101–1107
Sorbye H, Welin S, Langer SW et al (2013) Predictive and prognostic factors for treatment and survival in 305 patients with advanced gastrointestinal neuroendocrine carcinoma (WHO G3): the NORDIC NEC study. Ann Oncol 24:152–160
Vélayoudom-Céphise FL, Duvillard P, Foucan L, Hadoux J, Chougnet CN, Leboulleux S, Malka D, Guigay J, Goere D, Debaere T, Caramella C, Schlumberger M, Planchard D, Elias D, Ducreux M, Scoazec JY, Baudin E (2013) Are G3 ENETS neuroendocrine neoplasms heterogeneous? Endocr Relat Cancer 20:649–657
Rinke A, Müller H-H, Schade-Brittinger C et al (2009) 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 27:4656–4663
Caplin ME, Pavel M, Cwikla JB et al (2014) Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med 371:224–233
Raymond E, Dahan L, Raoul JL, Bang YJ, Borbath I, Lombard-Bohas C et al (2011) Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med 364(6):501–513
Yao JC, Shah MH, Ito T, Bohas CL, Wolin EM, Van Cutsem E et al (2011) Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med 364(6):514–523
Kwekkeboom DJ et al (2008) Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0, Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol 26(13):2124–2130
Sansovini M, Severi S, Ambrosetti A, Monti M, Nanni O, Sarnelli A, Bodei L, Garaboldi L, Bartolomei M, Paganelli G (2013) Treatment with the radiolabelled somatostatin analog Lu-DOTATATE for advanced differentiated neuroendocrine tumors. Neuroendocrinology 97(4):347–354. doi:10.1159/000348394, Epub 2013 May 22
Ezziddin S, Khalaf F, Vanezi M, Haslerud T, Mayer K, Al Zreiqat A, Willinek W, Biersack HJ, Sabet A (2014) Outcome of peptide receptor radionuclide therapy with 177Lu-octreotate in advanced grade 1/2 pancreatic neuroendocrine tumours. Eur J Nucl Med Mol Imaging 41(5):925–933. doi:10.1007/s00259-013-2677-3
Bodei L, Kidd M, Paganelli G, Grana C, Drozdov I, Cremonesi M, Lepenski C, Kwekkeboom D, Baum R, Krenning E, Modlin IM (2015) Long-term tolerability of PRRT in 807 patients with neuroendocrine tumours: the value and limitations of clinical factors. Eur J Nucl Med Mol Imaging 42(1):5–19
Strosberg JR, Fine RL, Choi J et al (2011) First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer 117:268–275
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Fazio, N. (2015). Medical Therapy of Pancreatic Neuroendocrine Neoplasms. In: La Rosa, S., Sessa, F. (eds) Pancreatic Neuroendocrine Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-17235-4_22
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DOI: https://doi.org/10.1007/978-3-319-17235-4_22
Publisher Name: Springer, Cham
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