Neuroendocrine Tumor Consensus Guidelines Released

Pancreas

Neuroendocrine tumors (NETs) are a group of rare cancers that pose unique challenges in diagnosis and treatment. In a recent issue of , official journal of the American Pancreatic Association, presents the newly developed consensus guidelines of the North American Neuroendocrine Tumor Society (NANETS).

The culmination of years of dedicated efforts by a host of worldwide experts, the NANETS consensus guidelines present an up-to-date synthesis of the best available knowledge on the classification, diagnosis, and treatment of NETs.

"Our hope is that these guidelines will provide the practical information necessary for professionals from a variety of specialties to reach a proper diagnosis and develop a treatment plan for their NET patients," write NANETS Chair Emeritus Larry K. Vols, MD, and Executive Director Kari L. Brendtro. "In particular, we anticipate that these will be useful resources for busy physicians who may only encounter these tumors infrequently."

Up-to-Date Guidelines on a Rare Group of Cancers

The diagnosed incidence of neuroendocrine tumors is increasing, and the overall prevalence in the United States has been estimated to exceed 100,000 individuals. Neuroendocrine tumors are often characterized by the ability to secrete different hormones, and can occur in most organs of the body, including the stomach and pancreas, intestines, thymus, and thyroid—anywhere there are "neuroendocrine" cells.

One of the more common types of neuroendocrine tumors are carcinoid tumors, which are sometimes associated with symptoms of flushing and diarrhea (the carcinoid syndrome). In addition to carcinoid tumors, other subtypes of NETs include pancreatic neuroendocrine tumors such as insulinomas, glucagonomas, and gastrinomas, as well as less common neuroendocrine tumors such as pheochromocytomas.

If detected early, NETs can often be treated successfully with surgery. Unfortunately, NETs can be present for a long time before symptoms appear. Many patients go for years before the correct diagnosis is made, by which time the cancer has spread and is no longer curable

Developing the consensus guidelines as a practical resource for medical management of NETs was the first task decided on after the formation of NANETS in 2006. Scientists, physicians, and surgeons—all with a special interest in NETs—were organized into working groups to address specific topics. A review process was developed to ensure that the guidelines represented a synthesis of the best available scientific and medical knowledge of these unfamiliar tumors.

Specific Guidance on NET Diagnosis and Treatment

The eight papers published in the August issue of Pancreas present an up-to-date overview of the different types of NETs and their clinical management. A key issue is a review of systems for classification of NETs, focusing on the essential information needed to clarify the exact type of tumor that is present. The NANETS guidelines present specific information on the diagnosis and management of the different types of NETs occurring in various locations.

The overriding goal is to help physicians and other health professionals recognize, as early as possible, the symptoms that suggest a possible diagnosis of NETand to provide patients with appropriate treatment for their malignancy.

The NANETS guidelines are accompanied by a "critical appraisal" of current treatments for NETs. Because NETs are rare, there have, to date, been relatively few FDA approved treatments for this indication. The critical review of available treatments accompanying the guidelines therefore represents another important resource for physicians and their patients.

Vay Liang W. Go, MD, Editor-in-Chief of Pancreas and member of the Executive Committee of NANETS, comments, "We are pleased to announce this affiliation, and are confident that will help to n further the ultimate goal of developing effective strategies that will improve quality of life and optimize survival for patients with NETs."