What are neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs)?
Neuroendocrine tumors (NETs) is an umbrella term for a group of rare tumors that can appear anywhere in the body where there are neuroendocrine cells. Neuroendocrine carcinomas (NECs) are an even rarer subset of NETs. NETs can be found in the lungs, stomach, pancreas, liver, small intestine, rectum, and other sites. In addition, NETs can be slow growing (“well differentiated” or “low proliferating”) with few symptoms to extremely fast-growing (“high grade” or “poorly differentiated”), malignant tumors that often metastasize to other organs. These high-grade NETs are sometimes referred to as Neuroendocrine Carcinomas (NECs). Symptoms may mimic other diseases, which in turn make diagnosis difficult. NETs are a group of cancers listed with the National Organization of Rare Disorders (NORD).
Are NETs and NECs rare?
Yes. See the following statistics:
An estimated 90% of NETs are initially incorrectly diagnosed.
Lung NETs make up approximately 1% to 2% of all lung tumors (with an estimated 2,000 to 4,500 adults diagnosed each year in the U.S with a form of lung NET)†† and make up roughly 25% of all NETs.
Neuroendocrine carcinomas (NECs) are even rarer, with fewer than 2,000 new cases diagnosed each year in the U.S.†††
† Neuroendocrine Tumors: Statistics, Approved by the Cancer.Net Editorial Board, 01/2020 (https://www.cancer.net/cancer-types/neuroendocrine-tumors/statistics)
†† Neuroendocrine Tumor of the Lung: Statistics, Approved by the Cancer.Net Editorial Board, 01/2020 (https://www.cancer.net/cancer-types/neuroendocrine-tumor-lung/statistics)
Breast cancer has the pink ribbon. Do NETs or NECs have their own symbol?
Yes! The zebra has become the international symbol for NETs. This came about because doctors are generally taught that when you hear hoofbeats, it must be a horse. Doctors are not taught to look out for zebras, which are rarer than horses and, in fact, unique in their stripe patterns. So, zebras have become symbolic representations for NETs as each is different.
Is a carcinoid tumor the same as a NET?
The term “carcinoid” is a bit dated. There was a time when doctors referred to NETs in different ways. You might hear terms like “typical carcinoid” or “atypical carcinoid” but for the most part, doctors on the forefront of NET treatment no longer use these labels (even though they remain in common usage). Rather, the type of NET/NEC and grade is a function of how the cancerous cells appear to a pathologist under a microscope (that is, “well differentiated” vs. “poorly differentiated”), the mitotic count of the cells, and the fraction of Ki-67-positive tumor cells.
Here are some helpful explanations on the current categorization of NETs and the history of why the term “carcinoid” was used to describe certain NETs:
Neuroendocrine Neoplasms Grade and Stage
Neuroendocrine Tumors Benign vs Malignant
Where can I learn more about NETs and NECs?
There are a lot of resources available. Here is a sampling of websites with information that might be helpful in learning more about NETs and NECs.
Carcinoid Cancer Foundation
Mission: The Carcinoid Cancer Foundation is the oldest nonprofit carcinoid/and related neuroendocrine tumor organization in the United States, founded in 1968. The mission of this foundation is to increase awareness and educate the general public and healthcare professionals regarding carcinoid and related neuroendocrine tumors (NETs), to support NET cancer patients and their families, and to serve as patient advocates.
Healing NET Foundation
Mission: The Healing NET Foundation mission is to optimize the care of those with neuroendocrine cancer through the education of and collaboration among physicians, health care providers, patients, and caregivers.
International Neuroendocrine Cancer Alliance (INCA)
Mission: INCA is the global voice for Neuroendocrine cancer (NET) patients and their carers with a mission to:
- raise awareness about all types of NETs;
- push for scientific advancements with a focus on identified unmet needs;
- and provide a platform for global collaboration to address the many challenges NET patients and the medical community face, in securing a timely diagnosis and accessing optimal treatment, support and care.
Los Angeles Carcinoid Neuroendocrine Tumor Society (LACNETS)
Mission: The mission of the Los Angeles Carcinoid Neuroendocrine Tumor Society (LACNETS), a program by Generate Possibility, is to provide a community of support and education for patients/those with neuroendocrine cancer and to advocate for all people impacted by this rare disease by expanding awareness and by voicing the patient’s perspective in collaborative dialogue with healthcare professionals. We aim to positively impact the quality of life of those with neuroendocrine cancer by offering support and expanded access to information on diagnosis, treatment options, research and resources. LACNETS provides a venue/makes it possible for patients to share experiences, encouragement and hope.
National Comprehensive Cancer Network
Mission: The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit alliance of 30 leading cancer centers devoted to patient care, research, and education. NCCN is dedicated to improving and facilitating quality, effective, efficient, and accessible cancer care so patients can live better lives.
National Institute of Health
Neuroendocrine Cancer Awareness Network (NCAN)
Mission: The Neuroendocrine Cancer Awareness Network (NCAN) is a non-profit organization dedicated to raising awareness of Neuroendocrine Cancer, providing support for caregivers and people with NETs, and funding for NET cancer research. Since 2003, it has been our mission, or as we like to say, our passion, to educate and support the NET community as a whole.
Neuroendocrine Tumor Research Foundation
Mission: The mission of the Neuroendocrine Tumor Research Foundation is to fund research to discover cures and more effective treatments for neuroendocrine cancers. The Neuroendocrine Tumor Research Foundation is committed to improving the lives of patients, families, and caregivers affected by neuroendocrine cancer by providing information and educational resources.
NJ Carcinoid Cancer Network
Mission: The New Jersey Carcinoid Cancer NETwork’s mission is to promote awareness of Carcinoid and other Neuroendocrine Tumors (NETs) and to provide shared information to patients, caregivers and medical professionals to assist them in managing their disease, and to provide treatment and diagnostic options that may not be common knowledge.
North American Neuroendocrine Tumor Society (NANETS)
Mission: The North American Neuroendocrine Tumor Society (NANETS) is a society of over 400 multidisciplinary medical professionals committed to the support of the medical community in treating neuroendocrine tumor (NET) patients, as well as furthering NET science and education in North America.
Northern California CarciNET Community
Mission: The Northern California CarciNET Community’s mission is to help those with neuroendocrine tumors and carcinoid to share challenges and experiences, learn about prognosis and treatments, find information, and improve communications between the medical community, patients and caregivers. By providing opportunities for our members to connect with others who have these rare illnesses we will enable them not only to get their questions answered but also to give and receive emotional support. In addition, we aspire to bring greater awareness to the medical community and the public so as to promote earlier detection and treatment of these often unrecognized illnesses.