The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Medical Management of Midgut Neuroendocrine Tumors

Abstract

There have been significant developments in diagnostic and therapeutic options for patients with neuroendocrine tumors. Key phase III studies include the CLARINET trial which evaluated lanreotide in patients with non-functioning enteropancreatic NETs, the RADIANT 2 and RADIANT 4 studies, which evaluated everolimus in functioning and non-functioning NETs of the GI tract and lungs, the TELESTAR study which evaluated telotristat ethyl in patients with refractory carcinoid syndrome, and the NETTER-1 trial which evaluated 177Lutetium-dotatate in NETs of the small intestine and proximal colon (midgut). Based on these and other advances, the North American Neuroendocrine Tumor Society (NANETS) convened a multidisciplinary panel of experts with the goal of updating consensus-based guidelines for evaluation and treatment of midgut NETs. The medical aspects of these guidelines (focusing on systemic treatment, nonsurgical liver-directed therapy, and post-operative surveillance) are summarized in this manuscript. Surgical guidelines are described in a companion manuscript.

Nomogram predicting the risk of recurrence after curative‐intent resection of primary non‐metastatic gastrointestinal neuroendocrine tumors: An analysis of the U.S. Neuroendocrine Tumor Study Group

“…Current recommendations for peri‐operative management (ie, analogues of somatostatin, neo‐adjuvant, and adjuvant chemotherapy) and post‐operative surveillance of patients who undergo curative‐intent surgery of primary GEP‐NET are largely based on expert opinion and small retrospective studies focused on specific subtypes of neuroendocrine tumors . Moreover, the optimal prognostic classification (ie, nomogram and staging system) to stratify risk of recurrence for GEP‐NET patients has not been defined.…”

Efficacy of Peptide Receptor Radionuclide Therapy in a United States–Based Cohort of Metastatic Neuroendocrine Tumor Patients

“…This question of where PRRT should be utilized continues to be a subject of debate in recent society guidelines addressing the treatment of pancreatic and midgut neuroendocrine tumors. 24,25 In our cohort we analyzed PFS and OS in patients who were treated with either non-hepatic surgery, liver directed therapy including hepatic resection, and/or systemic chemotherapy prior to PRRT. The total number of different treatments and having a non-hepatic surgery prior to PRRT did not affect PFS or OS in our analysis.…”

SATB2 in neuroendocrine neoplasms: strong expression is restricted to well‐differentiated tumours of lower gastrointestinal tract origin and is most frequent in Merkel cell carcinoma among poorly differentiated carcinomas

“…For example, pancreatic NETs are more likely to present with distant metastases than jejunoileal NETs, and are associated with inferior median survival. NET primaries are often resected to militate against local complications, even in the face of distant metastasis; and different biological and cytotoxic therapies are approved according to primary site . Our group frequently employs a panel of immunohistochemical stains in this diagnostic setting, including CDX2 (midgut); PAX6, Islet 1 (pancreas); and OTP (lung) .…”