By Anna Greene, PhD, NETRF Director of Research
A major new study published in JAMA Network Open by Arvind Dasari, MD, MS, and colleagues provides a comprehensive update on the state of neuroendocrine neoplasms (NENs), which include well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs), in the United States. The findings are striking. Analyzing over 145,000 cases across decades, this represents the most complete picture yet of how NENs are impacting Americans today.
Here’s what you need to know.
NENs Are Being Diagnosed More Often
Between 1975 and 2021, the number of diagnosed neuroendocrine neoplasms rose more than fivefold. In 1975, the incidence rate was just 1.64 per 100,000 people. By 2021, that number surged to 8.52. Most of this increase is due to a rise in localized (early-stage) NETs, many of which are found incidentally during scans or surgeries for other conditions.
Interestingly, this rise affects all ages, sexes, races, tumor grades, and primary tumor locations. However, the most significant increases are seen in tumors in the appendix and rectum, each growing 12-fold since 1975. NENs are also becoming more common in the pancreas, lung, and small intestine.
This rapid rise is likely due to improvements in diagnostic tools, more routine imaging, and greater awareness among physicians.
Survival Is Improving—Even for Advanced Cases
Here’s some good news: People with NENs are living longer than ever. The median overall survival for all NETs is now 16.8 years, though for NECs, there is more work to be done to increase survival (median overall survival is 10 months). For early-stage NETs in the appendix or rectum, median survival exceeds 30 years. Even for more serious, advanced cases, such as NETs that have spread to other parts of the body, survival has steadily improved, particularly in the last two decades.
This improvement is likely due to advancements in imaging, such as DOTATATE-PET, and improvements in treatments, including targeted therapies like everolimus and peptide receptor radionuclide therapy (PRRT), as well as, in some cases, cytotoxic chemotherapy, such as capecitabine/temozolomide (CAPTEM).
NENs May Be More Common Than Previously Thought
As of January 2021, an estimated 248,546 Americans were living with a neuroendocrine neoplasm diagnosis in the previous 20 years. That’s over 70,000 more than in 2017.
Yet despite this increasing number, certain subsets of NENs, like pancreatic NETs or lung NETs, are still categorized as rare diseases, which helps ensure ongoing support for orphan drug development. Many therapies used to treat NENs benefit from this designation, providing patients with greater access to innovative treatments.
What This Means for Patients and Families
If you or someone you love has been diagnosed with a NEN, take heart: survival is improving, research is advancing, and treatments are becoming more precise. But it’s important to understand your specific diagnosis, including the tumor’s location, grade, and stage.
This study emphasizes the importance of ongoing research. At NETRF, we are dedicated to funding innovative research to ensure continuous discoveries, so that the future offers better outcomes for individuals with neuroendocrine cancer.
Stay Informed, Stay Empowered
We encourage all patients, caregivers, and healthcare professionals to stay engaged with the latest science. Stay tuned to our blog for updates on the latest research.
Together, we’re advancing knowledge, improving care, and changing the story of neuroendocrine cancer—one discovery at a time.
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