The Evolution of Neuroendocrine Tumor Treatment Reflected by ENETS Guidelines

Abstract

In 2016, the third version of guidelines for the diagnosis and treatment of neuroendocrine tumors (NETs) has been published by the European Neuroendocrine Tumor Society (ENETS). These guidelines reflect the progress in treatment of NETs, and by comparing the newest guidelines with the first guidelines of 2001, this progress can be clearly recognized. Diagnostic accuracy has been increased by the introduction of PET-CT with Ga-labelled somatostatin analogs, and multiple new treatments and treatment schedules have been developed, like peptide receptor radiotherapy with radiolabeled somatostatin analogs, or targeted therapies. Evidence and indications for these therapies are discussed in the ENETS guidelines. In this review, we aim to show the progress in NET diagnosis and treatment on the basis of the advances in the guidelines, but also to discuss the unsolved questions and unmet needs which still remain.

Prognostic value of a three-scale grading system based on combining molecular imaging with 68Ga-DOTATATE and 18F-FDG PET/CT in patients with metastatic gastroenteropancreatic neuroendocrine neoplasias

“…Traditionally, 18 F-FDG PET has been used for staging purposes in poorly-differentiated NECs or in case of negative SSTR imaging [16,17], as poorlydifferentiated NENs lose their ability to express SSTR. However, several prospective data underlined its strong independent prognostic value in NENs, even in those with low histological grade.…”

Duodenal neuroendocrine tumors: Somewhere between the pancreas and small bowel?

“…Although the majority are largely indolent, the biologic behavior of GEP‐NETs and their associated outcomes vary widely based on anatomic location and size of the primary tumor. To address the challenges that arise from the heterogeneity of these tumors, both the European Neuroendocrine Tumor Society (ENETS) and the North American Neuroendocrine Tumor Society (NANETS) have developed consensus guidelines for the diagnosis and management of GEP‐NETs based on primary tumor site . Although initial guidelines considered duodenal neuroendocrine tumors (D‐NETs) as a subset of small bowel GEP‐NETs, updated guidelines acknowledge that D‐NETs behave biologically different and are subject to different staging and management recommendations.…”

p.L105Vfs mutation in a family with thymic neuroendocrine tumor combined with MEN1: a case report

“…As for clinical management of NTEs, the European Society for Medical Oncology (ESMO), the European Neuroendocrine Tumor Society (ENETS) and the National Comprehensive Cancer Network (NCCN) Guidelines recommend that surgical resection is the main therapy for localized disease, and also a choice for extensive disease. External radiotherapy, cytotoxic treatment and Interferon-Alpha can be used when disease progresses [30][31][32]. Besides, according to the ENETS and NCCN Guidelines [31,32], treatment with Octreotide or Lanreotide, peptide receptor radionuclide therapy (PRRT) or targeted therapy (Everolimus) are recommended for advanced disease.…”

“…External radiotherapy, cytotoxic treatment and Interferon-Alpha can be used when disease progresses [30][31][32]. Besides, according to the ENETS and NCCN Guidelines [31,32], treatment with Octreotide or Lanreotide, peptide receptor radionuclide therapy (PRRT) or targeted therapy (Everolimus) are recommended for advanced disease. ENETS [31] also recommend Interventional Radiology for selected NETs patients with high liver burden.…”

“…Besides, according to the ENETS and NCCN Guidelines [31,32], treatment with Octreotide or Lanreotide, peptide receptor radionuclide therapy (PRRT) or targeted therapy (Everolimus) are recommended for advanced disease. ENETS [31] also recommend Interventional Radiology for selected NETs patients with high liver burden. In this case, the proband underwent thymic resection, chemotherapy and Lanreotide therapy, and now he is still alive.…”