A Comparison Between Appendiceal and Nonappendiceal Neuroendocrine Tumors in Children and Young Adults

Abstract

Well-differentiated appendiceal tumors were the most common pediatric NET and have an excellent prognosis. Better therapies are needed for patients with nonappendiceal NET.

Systematic review and meta‐analysis of appendiceal carcinoid tumors in children

“…The experience seems to be different in children as studies in children suggest appendiceal carcinoids are benign with excellent clinical outcomes . The 5‐year recurrence survival and the overall survival have been reported as 100% in children …”

“…6,7 The 5-year recurrence survival and the overall survival have been reported as 100% in children. 15 Several pediatric studies have questioned the use of these guidelines in children and the benefit of more extensive procedures like RHC following the incidental histological diagnosis of an appendiceal carcinoid. 2,6,8,9,16 Carcinoid syndrome from an appendiceal carcinoid has not been described in children.…”

Surgical Management of Neuroendocrine Tumors of the Appendix in Children and Adolescents: A Retrospective French Multicenter Study of 114 Cases

“…Deaths in adult patients were related to distant metastatic spread at presentation . Most series report the absence of recurrence and fatalities with a follow‐up up to 35 years irrespective of the type of surgery—simple appendectomy or RHC . Syracuse et al.…”

“…[3,6,11] Most series report the absence of recurrence and fatalities with a follow-up up to 35 years irrespective of the type of surgery-simple appendectomy or RHC. [15,17,22,28] Syracuse et al published the natural history of these tumors, and went over surgical cases at one center for over 70 years and found no deaths related to NET of the appendix. [9] Most authors conclude that these tumors have no influence on overall or event-free survival and appendectomy alone appears to be curative.…”

A 17-year-old male with a Small Bowel Neuroendocrine Tumor: flushing differential diagnosis

“…NETs are heterogeneous malignant neoplasms, which originate from cells with a secretory function within the neuroendocrine system [ 1 ]. The median age of diagnosis is 66 years-old [ 2 ] and it tends to be more common in females [ 3 – 6 ]. NETs are uncommon in the pediatric population with an incidence around 0.995 cases per 100,000 in patients under 20 years old [ 3 ].…”