Neuroendocrine Tumors in Pediatrics

Abstract

Neuroendocrine cells are dispersed diffusely throughout many organ systems in the body and hence neuroendocrine tumors (NETs) can arise from almost anywhere in the body. NETs are considered rare tumors, and the current incidence is reported to be about 6 cases in 100 000 in adults and about 2.8 cases per million in the pediatric age group. Despite the indolent nature of these tumors, they have the potential for metastasis and significant morbidity. NETs can be asymptomatic at the time of diagnosis or can present with flushing, diarrhea, wheezing, weight loss, and fatigue among other symptoms. Due to the ambiguity of presenting symptoms, it is not uncommon for NETs to be diagnosed late in the disease course. Despite low incidence, the prevalence of the disease is high since patients live for many years and sometimes decades. Early detection of well-differentiated NETs has excellent outcomes with the majority of early-stage diseases being cured with surgical resection alone. There have been recent advancements in the management of metastatic progressive NETs with approval of peptide receptor radionuclide therapy, telotristat, and everolimus. Awareness of these rare tumors and its management is crucial for optimal management. This article will focus on pediatric NETs and current advances in its management.

Childhood neuroendocrine tumors of the digestive system

“…[55] Appropriate and prudent treatment approaches can prevent recurrence and ensure diseasefree survival with increased awareness of NETs in pediatric patients. Early diagnosis is crucial because surgical resection can be curative [12] and strategies to increase awareness of the disease in pediatric patients, discovering therapeutic treatments, and sharing experiences are critical for developing an optimal treatment modality for this population.…”

“…The recently developed PET/CT imaging technique with radiolabeled somatostatin analogs is used as the new gold standard [30] . Studies suggest that 68 Ga-DOTATATE PET/CT should be considered a first-line diagnostic tool in adult and pediatric patients [12] . 68 Ga-DOTATATE is superior to most imaging techniques, has low exposure to radiation, low toxicity, fast administration/clearance time, and cost-effectiveness.…”

“…[11] Recent studies have indicated the increased prevalence and incidence of NETs over the last few decades, which could result from improved diagnostics and increased detection rates of the disease. [12] In this study, patients with gastrointestinal neuroendocrine tumor (GI-NET) followed up and treated in our center and also a patient with a very rare disease (primary hepatic NET) who presented with endocrinological symptoms were reviewed in the light of current literature.…”

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

“…[ 4 ] Neuroendocrine tumors can occur in many types of organs since neuroendocrine cells are distributed throughout various organs in the human body. [ 5 ] Neuroendocrine tumors are usually more common in adults than in children. Although the incidence of neuroendocrine tumors is low in children, with approximately 2.8 cases per million, the rate is steadily increasing.…”

Peptide Receptor Radionuclide Therapy with [177Lu]Lu-DOTA-TATE in Patients with Advanced GEP NENS: Present and Future Directions

“…Although overall incidence and prevalence of GEP-NETs has been gradually increasing over the last few decades, data on the pediatric population specifically are limited due to its rare occurrence in this population [57]. NETs in children show a high SSTR expression and could be potentially treated with PRRT-Lu; however, children under the age of 18 have been excluded from participation on PRRT trials.…”