Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability

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.

Safety and Efficacy of 177Lu-DOTATATE in Children and Young Adult Population

“…3 Moreover, due to their indolent nature and associated vague signs and symptoms, there is a delay in the diagnosis of NETs by about 10 years, thus indicating tumorigenesis at an early age, even in young adults. 15 Flushing, abdominal pain, diarrhea, and cough often occur in children and young adults, thus increasing uncertainty in diagnosing NETs and often leading to misdiagnosis. 3,15,16 Of all the patients evaluated for a syndromic association in our cohort, 2 had MEN-1 syndrome, and 1 each had MEN-2A, and neurofibromatosis type 1.…”

“…15 Flushing, abdominal pain, diarrhea, and cough often occur in children and young adults, thus increasing uncertainty in diagnosing NETs and often leading to misdiagnosis. 3,15,16 Of all the patients evaluated for a syndromic association in our cohort, 2 had MEN-1 syndrome, and 1 each had MEN-2A, and neurofibromatosis type 1. Clinical screening did not reveal a familial syndrome in the rest of the patients.…”

“…Only 15% of patients with MEN-1 are diagnosed in childhood and require lifelong biochemical and radiological screening. 15,17,18 The Surveillance, Epidemiology, and End Results (SEER) data on the analysis of NETs in the 0-29 years age group revealed that the appendix, lung, and breast are the more common sites of primary NET in the pediatric and young adult population. 3 However, from our institute, serving the population residing in northern India, the pancreas was found to be the most common site (64% of patients) of primary NET in this age group, concordant with previously published Indian registry data on adults.…”

“…NETs are rare in children and young adults, with an incidence of 2.8 per million and a prevalence of only 7724 3 . Moreover, due to their indolent nature and associated vague signs and symptoms, there is a delay in the diagnosis of NETs by about 10 years, thus indicating tumorigenesis at an early age, even in young adults 15 . Flushing, abdominal pain, diarrhea, and cough often occur in children and young adults, thus increasing uncertainty in diagnosing NETs and often leading to misdiagnosis 3,15,16 .…”

Young-Onset Cancers—Early Steps in the Right Direction

“…The issue also features two review articles by Castle and colleagues [9] on paediatric neuroendocrine neoplasms and Dudzisz-Śledź and colleagues on the treatment of gastrointestinal stromal tumours in younger patients [10].…”