Childhood neuroendocrine tumors of the digestive system: A single center experience

Abstract

The prevalence and incidence of neuroendocrine tumors (NETs) are increasing in the pediatric population. This increase can be associated with improved diagnostics and increased detection rates of the disease. We aimed to discuss the clinical and pathological characteristics of patients with this rare disease who were followed and treated at our center.The medical records of children (aged 0-18 years) with NETs of the digestive system, followed up and treated between 2007 and 2020 at Ondokuz Mayis University Faculty of Medicine, were reviewed.Overall, 16 patients (8 girls and 8 boys) were analyzed. Fifteen patients had NETs in the appendix; 14 of these had grade I NETs, and 1 had grade II NETs. No additional surgery was performed except for appendectomy. All patients were in complete remission at the last follow-up (median 38 months). The other patient, a 12-year-old girl, had a primary hepatic neuroendocrine carcinoma (grade III NET). Three cycles of neoadjuvant and adjuvant platinum-based chemotherapy were administered, and right hepatectomy was performed to remove the mass. The patient is being followed-up for approximately 3 years without disease recurrence.Most NETs are observed in adults, and most studies have focused on this population. Unlike adults, increasing awareness of the disease in the pediatric population (especially in cases of acute appendicitis), discovering therapeutic treatments, and sharing experiences are crucial for developing an optimal therapeutic approach for pediatric NETs.

Figure 1

Clinical signs of the patient at the time of admission.

Figure 2

Face image before and after treatment.

Figure 3

Abdominal MR image of the mass at the time of diagnosis.