Priorities for improving the management of gastroenteropancreatic neuroendocrine tumors

Abstract

A National Cancer Institute summit meeting on gastroenteropancreatic neuroendocrine and carcinoid tumors was held in September 2007 to present the currently accepted standards of care for patients with these tumors and to identify areas requiring investigation and development. These tumors are clinically and pathologically heterogeneous, present commonly with obscure symptoms that lead to delays in diagnosis of years, and have an incidence in the United States of 2.5 to 5 cases per 100,000. The 5-year survival rates range between 15% and 95%, depending on the site and extent of disease. This report delineates the main conclusions of the meeting, including the best practice diagnosis and treatment strategies for gastropancreatic neuroendocrine tumors, and the identification of clinical and scientific areas that are most in need of attention. The most pressing needs were public and physician education, identification of molecular markers for early diagnosis and therapeutic monitoring, improved imaging modalities and molecular prognostication, development of a standardized pathological classification system, and creation of regional centers of expertise with tumor and laboratory data banks. In addition, adequately validated neuroendocrine tumor models and cell lines should be established to investigate the molecular mechanisms involved in the control of their growth and secretion, and to facilitate the development of specific therapies that should be examined in well-designed multicenter studies of defined patient groups.

Figure 1

Increasing incidence of gastroenteropancreatic neuroendocrine tumors in the US population, 1973–2005. A) Current and estimated future incidence of gastroenteropancreatic neuroendocrine tumors in the United States. The yearly incidence of gastroenteropancreatic neuroendocrine tumors registered in the US National Cancer Institute Surveillance, Epidemiology and End Results (SEER) database 1983–2003 is shown in green. Regression analysis of the calculated increase in incidence 2003–2013 is shown in red. B) The incidence of neuroendocrine tumors by anatomical location from 1973 through 2004 (SEER database). The range of overall increase is 221%–626%.

Figure 2

Pie chart of National Institutes of Health disease funding of research in 11 types of cancer, in billions of US$, 1994–2002.