Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability

SIMPLE SUMMARY: Neuroendocrine neoplasms are increasing in incidence at a remarkable rate meaning more providers are encountering them in both adult and pediatric patients. This classification of neoplasm encompasses a wide range of different malignancies with a variety of symptoms at presentation and each treated differently. Additionally, over the past few years there has been a change in classification of these neoplasms and a variety of changes and advances in how they are treated. Given this and their rarity in pediatric patients, healthcare providers may not be familiar with these changes. Our goal with this review was to provide an overview of all the most commonly encountered forms of neuroendocrine neoplasms in pediatric patients with up to date recommendations so any healthcare provider can quickly and accurately acclimate themselves. ABSTRACT: Neuroendocrine neoplasms (NENs) encompass a variety of neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) which can arise anywhere in the body. While relatively rare in the pediatric population, the incidence of NENs has increased in the past few decades. These neoplasms can be devastating if not diagnosed and treated early, however, symptoms are variable and can be indolent for many years. There is a reported median of 10 years from the appearance of the first symptoms to time of diagnosis. Considering some of these neoplasms have a mortality rate as high as 90%, it is crucial healthcare providers are aware of NENs and remain vigilant. With better provider education and easily accessible resources for information about these neoplasms, awareness can be improved leading to earlier disease recognition and diagnosis. This manuscript aims to provide an overview of both the most common NENs as well as the rarer NENs with high lethality in the pediatric population. This review provides up to date evidence and recommendations, encompassing recent changes in classification and advances in treatment modalities, including recently completed and ongoing clinical trials.