Abstract

The systemic treatment of patients with pancreatic neuroendocrine tumors is based on placebo-controlled trials and long-established chemotherapy approaches. In addition, peptide receptor radionuclide therapy (PRRT) was approved as a parallel approach for pancreatic neuroendocrine tumors (NET), in addition to small bowel NET, after the NETTER-1 trial. The current ESMO and NCCN guidelines attempted to describe treatment algorithms for pancreatic NET based on the current data. In our survey, we recorded therapy decisions for the first- until the third-line of therapy in German-speaking countries (Germany, Austria, and Switzerland) using fictional case reports and discussed these in the context of the current ESMO guidelines. Compared with the recommendations of the guidelines, PRRT was used more frequently and earlier. In patients with NET G1/G2 Ki-67 < 10%, the therapy algorithm consisting of somatostatin analogs (SSA)-PRRT-targeted therapy is a relevant approach. In clinical situations where chemotherapy is primarily used (remission pressure, Ki-67 > 10%), second-line PRRT was found acceptance and was often considered prior to targeted therapies. Despite the lack of prospective controlled trials, our study demonstrated the pivotal impact of PRRT. Therefore, further studies should compare PRRT with chemotherapy in pancreatic NETs in different clinical settings in first- and second-line approaches.

Highlights

  • Gastroentero-pancreatic neuroendocrine tumors (GEP-NETs) comprise a broad clinical and therapeutic spectrum of malignancies

  • A total of 32 questions were developed, including four general questions regarding the characteristics of the physicians and facilities, 14 case presentations, and a respective reasoning question to evaluate the management of pancreatic NET (PanNET) patients

  • The survey was performed between 1 July and 31 December 2020 in the following three German-speaking countries: Austria, Switzerland, and Germany

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Summary

Introduction

Gastroentero-pancreatic neuroendocrine tumors (GEP-NETs) comprise a broad clinical and therapeutic spectrum of malignancies. The spectrum of therapies has expanded significantly. In addition to the already established therapies of somatostatin analogs (SSA), locoregional liver-specific therapies such as TACE/SIRT, and chemotherapy for pancreatic NET, the targeted therapies everolimus/sunitinib have been approved for pancreatic and peptide radioreceptor therapy (PRRT) for all somatostatin receptor-positive GEP-NETs [2,3,4,5]. The NETTER-1 trial of PRRT in midgut NETs introduced a treatment protocol that both defined a fixed number of cycles and radiation doses and established the continuous use of SSA during therapy [5]. In addition to the numerous national guidelines for neuroendocrine neoplasms [6,7,8], the American (NCCN) and European (ENETS and ESMO) societies have launched the current guidelines that define comprehensive recommendations for the various therapeutic approaches [9,10,11]

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