Expert Consensus Practice Recommendations of the North American Neuroendocrine Tumor Society - Guidelines and Guidance - management of high grade gastroenteropancreatic and gynecologic neuroendocrine neoplasms

Sep 24, 2026

High-grade neuroendocrine neoplasms are rare, representing about 10% of all neuroendocrine neoplasms, and the limited availability of prospective data leaves clinicians relying heavily on expert opinion. Increasing complexity stems from distinguishing well-differentiated high-grade (G3) neuroendocrine tumors from poorly differentiated neuroendocrine carcinomas. The North American Neuroendocrine Tumor Society convened an expert panel to create consensus recommendations and a treatment algorithm for management and surveillance across gastrointestinal, pancreatic, and gynecologic systems.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages27

Reading Time1 h 8 min

AccessFree to download

Abstract

Introduction

Epidemiology and rarity

Pathologic classification complexity

Common primary sites

Prognosis differences and evidence gaps

| Endocrine-Related Cancer | J R Eads et al. | | 30:8 | e220206 | | --- | --- | --- | --- | --- | | GUIDELINES AND GUIDANCE
Expert Consensus Practice Recommendations of the North American Neuroendocrine Tumor Society for the management of high grade gastroenteropancreatic and gynecologic neuroendocrine neoplasms
Jennifer R Eads1, Thorvardur R Halfdanarson2, Tim Asmis3, Andrew M Bellizzi4, Emily K Bergsland5, Arvind Dasari6, Ghassan El-Haddad7, Michael Frumovitz8, Joshua Meyer9, Erik Mittra10, Sten Myrehaug11, Eric Nakakura12, Nitya Raj13, Heloisa P Soares14, Brian Untch15, Namrata Vijayvergia16 and Jennifer A Chan17
1Division of Hematology and Oncology, Abramson Cancer Center, University of Pennsylvania, Pennsylvania, USA
2Division of Medical Oncology, Mayo Clinic Cancer Center, Rochester, Minnesota, USA
3Division of Medical Oncology, University of Ottawa, Ottawa, Ontario, Canada
4Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA
5Department of Medicine, University of California, San Francisco, California, USA
6Division of Gastrointestinal Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
7Department of Diagnostic Imaging and Interventional Radiology, Moffitt Cancer Center and Research Institute, Tampa, Florida, USA
8Division of Gynecologic Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
9Department of Radiation Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
10Division of Molecular Imaging and Therapy, Oregon Health & Science University, Portland, Oregon, USA
11Department of Radiation Oncology, Odette Cancer Centre, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
12Department of Surgery, University of California, San Francisco, California, USA
13Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
14Division of Oncology, Huntsman Cancer Institute, University of Utah, Salt Lake City, Salt Lake City, Utah, USA
15Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA
16Department of Hematology and Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
17Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA
Correspondence should be addressed to J Eads: [jennifer.eads@pennmedicine.upenn.edu](jennifer.eads@pennmedicine.upenn.edu) | | | | | Abstract High-grade neuroendocrine neoplasms are a rare disease entity and account for approximately 10% of all neuroendocrine neoplasms. Because of their rarity, there is an overall lack of prospectively collected data available to advise practitioners as to how best to manage these patients. As a result, best practices are largely based on expert opinion. Recently, a distinction was made between well-differentiated high-grade (G3) neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas, and with this, pathologic details, appropriate imaging practices and treatment have become more complex. In an effort to provide practitioners with the best guidance for the management of patients with high-grade neuroendocrine neoplasms of the gastrointestinal tract, pancreas, and gynecologic system, the North American Neuroendocrine Tumor Society convened a panel of experts to develop a set of recommendations and a treatment algorithm that may be used by practitioners for the care of these patients. Here, we provide consensus recommendations from the panel on pathology, imaging practices, management of localized disease, management of metastatic disease and surveillance Key Words f neuroendocrine tumors f neuroendocrine carcinoma f recommendations f high-grade | Endocrine-Related Cancer | J R Eads et al. | | 30:8 | e220206 | | --- | --- | --- | --- | --- | | and draw key distinctions as to the approach that should be utilized in pa

FAQs

Why are consensus recommendations needed for high-grade neuroendocrine neoplasms?

High-grade neuroendocrine neoplasms are rare, and there is a lack of prospectively collected data to guide optimal diagnosis and management, so best practices have relied on expert opinion.

What key distinction does the document highlight within high-grade disease?

It distinguishes well-differentiated high-grade (G3) neuroendocrine tumors from poorly differentiated neuroendocrine carcinomas, making pathology details, imaging practices, and treatment more complex.

How do prognosis and survival differ between metastatic G3 neuroendocrine tumors and neuroendocrine carcinomas?

The prognosis for metastatic G3 NET is described as fair, averaging around 42 months, while neuroendocrine carcinomas have a poorer prognosis with median overall survival being less than a year, especially for primaries outside the small bowel and appendix.

Sep 24, 2026

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