The Dethroning of Neuroendocrine Neoplasms as an Orphan Disease: US Incidence, Prevalence, and Survival in the 21st Century

USA – Prevalence of Neuroendocrine Neoplasms (NENs) breaches the Orphan Disease threshold for the first time (officially)

The latest US SEER figures confirm that staggering increase in the prevalence of NENs. In that cross-sectional study which evaluated 145,477 NEN cases in the US, age-adjusted incidence rates increased 5.2-fold between 1975 and 2021, with an annual percentage change of 3% between 2000 and 2020, and the 20-year limited duration prevalence projected in the US population on January 1, 2021, was 243 896. Survival for all NENs improved, including for patients with distant-stage gastrointestinal and pancreatic NENs. And even this figure is understated as they have excluded some Neuroendocrine Carcinomas from the list adding to the known registry issues which must still need some work. Worth noting that although the data was published in 2025, the period covered was completed in 2021, meaning another 4 years of diagnoses has since passed – it must be assumed the figures are now even higher. I published those results in my running commentary blog – Click here to read more

I said on my Facebook page that this data would trigger a whole new discussion. I’ve even triggered some new posts myself – click here to read my own attempt at the impact – but from an advocacy perspective – Reframing Neuroendocrine Neoplasms beyond “Rare”

But one big take of the SEER data came out shortly after and features the same doctor who I quote in the graphic above. It is titled Dethroning of Neuroendocrine Tumor as an Orphan Disease: US Incidence, Prevalence, and Survival in the 21st Century and cited below:

Yu Q, Cao F, Hosein P, Huang B, Pinheiro PS, Wang Y, Del Rivero J, Lopes G, Chauhan A. Dethroning of Neuroendocrine Tumor as an Orphan Disease: US Incidence, Prevalence, and Survival in the 21st Century. Cancers (Basel). 2025 Oct 15;17(20):3323. doi: 10.3390/cancers17203323. PMID: 41154379; PMCID: PMC12563563.

Some of the content is interesting starting with an ‘important’ introduction “Neuroendocrine tumors (NETs) have traditionally been considered rare (orphan) diseases; however, improvements in diagnostic methods and heightened awareness about NETs may have altered their epidemiologic profile in the U.S”. I think they are using NET and NEN interchangeably in this paper. The conclusion is also worth emphasising “NETs are no longer orphan diseases based on current U.S. incidence and prevalence trends. With increasing survival and patient numbers, there is an urgent need for expanded multidisciplinary NET care and research efforts focused on survivorship and quality of life”.

The urgent need statement within that conclusion is very relevant and I also covered those points in my own patient view above. It is now clear the way advocacy organisations work must also rise with the up to date figures coming out of major countries such as US, UK and Australia. But it’s a fascinating add-on to the SEER study above and I enjoyed reading it – I hope you do too. Click here.

Why I’m not surprised by these figures?

1. I’ve been saying this for 10 years and so have many NET specialists including in USA.

2. In 2023, I also published a quote from the American Society of Clinical Oncology (ASCO) Post which clearly said ““As a result of perceived disease rarity and non-malignant clinical course, NENs have not been subjected to rigorous clinical research, as compared with other similar tumor types”, adding that they are uncommon but not rare. So I upped my game while most other members of the community continued with zoology themes.

Ronny

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