Why education and raising awareness of neuroendocrine tumors is so critical.

  1. YES, IT’S CANCER--”Carcinoid” or “carcinoma-like” was the term used by the researcher who first discovered this class of tumors. In 2000 the World Health Organization designated “carcinoid” and other rare tumors of the hormone-producing cells as “neuroendocrine tumors” (NETs). The original term has caused confusion and a misunderstanding persisting today that the tumors are benign.

  2. HOW RARE IS IT?--Neuroendocrine cancer is considered a rare cancer, which makes diagnosis more difficult, but it’s estimated that over 170,000 people are living with the disease in the U. S. A recent study showed an increase in the incidence from 1 in 100,000 in 1973 to 7 in 100,000 in 2012, possibly due to better detection.

  3. DIFFERENT IN EACH PATIENT--The cancer can be anywhere there are endocrine or hormone-producing cells, such as the lung, small intestine, pancreas, rectum, stomach, and other sites. Some tumors are only found incidentally because patients are asymptomatic, but other patients may have significant symptoms.

  4. DIFFICULT TO DIAGNOSE--Symptoms, such as obstruction in the digestive or respiratory tract or hormone-induced symptoms including diarrhea and flushing, can mimic other diseases like asthma, IBS, or Crohn’s. Medical students are taught to think “horses,” or typical diseases, when they “hear hoof beats,” rather than to think “zebras,” or exotic diseases, as they assess symptoms. Physicians need to suspect a “zebra” when a patient’s symptoms do not improve. Patients often go 5-7 years without a correct diagnosis.

  5. MANAGED BUT RARELY CURED--Neuroendocrine tumors often metastasize to sites such as the liver, lungs, bone or heart. Because many are slow-growing, they can take years to show themselves. There is a risk of both under-treating the disease by not recognizing its full extent and over-treating in particular cases where the tumors are very slow to grow.

  6. NOT ALL ARE SLOW-GROWING--Though the typical neuroendocrine tumor is slow-growing, there are more rare types that are highly aggressive and are referred to as “high-grade” or “poorly differentiated.” These patients are often treated more quickly and aggressively, including with chemotherapy, and long-term progression-free survival is rare.

  7. MULTI DISCIPLINARY TEAM--Patients benefit the most from a multi-disciplinary team, which can include a surgeon, gastroenterologist or pulmonologist, oncologist, pathologist, endocrinologist, radiologist, cardiologist, a nurse or nurse practitioner, and preferably a nutritionist. It is highly recommended that a NET specialist be part of or preferably lead the team. Currently in the United States there are no established criteria to guide the patient as to who can deliver the specialized care a NET patient needs.

  8. NEW TREATMENTS AND DIAGNOSTIC TOOLS--Since June 2016, the FDA has approved a new diagnostic test (Netspot, Gallium 68), new medication (Xermelo, telotristat ethyl), and a nuclear therapy (Lutathera, a form of PRRT) that have changed the landscape for better diagnosis and treatment of NET cancer. Physicians need to know about these new tools that are available.

  9. NEW CLINICAL TRIALS--Hundreds of trials are available and actively recruiting patients, but physicians not familiar with the disease do not know to recommend patients, and patients can find the navigation of available trials difficult. Search at Trials Today or visit https://clinicaltrials.gov/ and type “neuroendocrine” in the search box. Become a volunteer at ResearchMatch so a clinical trial can find you.

  10. INSURANCE REIMBURSEMENT--Like any patients with a chronic disease, NET patients often find themselves battling for coverage of scans, medication, and treatments because of the newness and expense of some tools and treatments as well as all the awareness and education obstacles stated above.