As a group of Cancers, I personally don’t believe Neuroendocrine based Cancer is as rare as we are led to believe, particularly when you include poorly differentiated types such as small cell lung cancer. According to the World Health Organisation (WHO), each year an estimated 400,000 children and adolescents from 0-19 years old develop cancer. Whether that is rare within individual countries, a particular geographical area, or related to a type of cancer, will depend on epidemiological data.  The WHO also confirms that cancer is a leading cause of death for children and adolescents as defined above.  The most common types of childhood cancers include leukemias, brain cancers, lymphomas, and solid tumours, such as neuroblastoma and Wilms tumours.  The first two cancers listed account for more than half of childhood cancers. 

When you look at the average age of a Neuroendocrine Cancer patient, you can be excused for thinking that it’s a disease confined to those of a ‘certain age’. However, there is a pediatric component of the disease with one US study suggesting around 3 per million in NET, which is rare.  I very much suspect it is higher than that as it isn’t clear if neuroblastomas are included (some studies include Neuroblastomas). I did note a study from the Netherlands indicated a rate of 5.4 per million. Additionally, different studies may be using different age groups (in one study, the highest end of the range was 30, in others 19). Plus, the latest Neuroendocrine Neoplasms WHO classification of tumours may encompass a wider grouping since the figures above were published.  Additionally, as the term “NET” is used it may not, therefore, include any poorly differentiated types and data is scarce to confirm.  Clearly, the Neuroendocrine scientific community need to provide the frameworks and criteria for common reporting to get the true epidemiological data. 

The most common primary sites of NET associated with childhood and adolescents would appear to be lung and appendix NETs, the latter appears to be quite commonly found after presenting at the ER with appendicitis pain.  In fact, I do note in my patient group that appendiceal NET patients in general are mostly younger than the average.  The other area where we see children and adolescents is in the familial grouping of Multiple Endocrine Neoplasia (MEN1 and 2a/2b) including Pheochromocytoma/Paraganglioma, Von Hippel–Lindau (VHL) disease, neurofibromatosis (NF) 1 and Carney complex. 

Finally, a plea.  I’d like to include a childhood section on my well-thumbed “Where to find a Neuroendocrine Cancer specialist” blog post, so please pass me your recommendations.  Many thanks in advance. 

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