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Review
. 2020 Jun 15;9(6):1860.
doi: 10.3390/jcm9061860.

Medical Treatment of Advanced Pancreatic Neuroendocrine Neoplasms

Affiliations
Review

Medical Treatment of Advanced Pancreatic Neuroendocrine Neoplasms

Lola-Jade Palmieri et al. J Clin Med. .

Abstract

Pancreatic neuroendocrine neoplasms (panNENs) are relatively rare but their incidence has increased almost sevenfold over the last four decades. Neuroendocrine neoplasms are classified according to their histologic differentiation and their grade. Their grade is based on their Ki-67 proliferation index and mitotic index. Their prognosis is highly variable according to these elements and treatments also vary according to their classification. Surgery is the only curative treatment for localized and advanced panNENs and offers a better prognosis than non-surgical treatments. In the case of an advanced panNEN without the possibility of resection and/or ablation, medical treatment remains the cornerstone for improving survival and preserving quality-of-life. PanNENs are considered as chemosensitive tumors, unlike midgut neuroendocrine tumors. Thus, panNENs can be treated with chemotherapy, but targeted therapies and somatostatin analogs are also treatment options. The scarcity and heterogeneity of NENs make their management difficult. The present review aims to clarify the medical treatments currently available for advanced panNENs, based on their characteristics, and to propose a treatment algorithm.

Keywords: chemotherapy; neoadjuvant; palliative; pancreatic neuroendocrine carcinoma; pancreatic neuroendocrine neoplasm; pancreatic neuroendocrine tumor; targeted therapy.

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Conflict of interest statement

L.-J.P. has conflicts of interest with Servier, Amgen, Merck, MSD, Keocyt; S.D. has conflicts of interest with Mylan, Amgen; C.B. has conflicts of interest with Novartis, Ipsen, Amgen, Bayer. R.C. has conflicts of interest with Bayer, Amgen, AAA, Keocyt, Ipsen, Novartis, Servier. A.B., M.D., A.-S.C. have no conflicts of interest.

Figures

Figure 1
Figure 1
Algorithm for treating advanced pancreatic neuroendocrine tumors. 5FU: 5-fluorouracil; CAP: capecitabine; NEC: neuroendocrine carcinoma; NET: neuroendocrine tumor; PRRT: peptide receptor radionuclide therapy; SD: stable disease; SSA: somatostatin analogs; STZ: streptozotocin; TEM: temodal.
Figure 2
Figure 2
68Ga-DOTATOC transaxial fusion image in color scale of a 43-year-old patient with hepatic lesions identified as grade 2 (Ki 18%) neuroendocrine tumor. 68Ga-DOTATOC identified a pancreatic lesion and several hepatic metastases. Given its proliferation index, the patient was treated with doublet chemotherapy with temodal and capecitabine.

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