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

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 and draw key distinctions as to the approach that should be utilized in patients with well-differentiated G3 neuroendocrine tumors vs poorly differentiated neuroendocrine carcinomas.

Automated summary

High-grade neuroendocrine neoplasms, accounting for 10% of all neuroendocrine neoplasms, are rare. Due to limited data, best practices for managing these patients are largely based on expert opinion. A panel of experts from the North American Neuroendocrine Tumor Society developed recommendations and a treatment algorithm to provide guidance for managing patients with high-grade neuroendocrine neoplasms. The consensus recommendations cover pathology, imaging practices, management of localized and metastatic disease, surveillance, and distinguish approaches for well-differentiated G3 neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas.