TY - JOUR
T1 - Management and outcome of high-risk neuroendocrine tumors of the appendix in children; A systematic review
AU - Raphael, M.F.
AU - van Amstel, Paul
AU - Mahieu, Amber
AU - Bakx, Roel
AU - de Vries, Ralph
AU - Derikx, Joep P. M.
AU - van Heurn, L. W. Ernest
AU - Gorter, Ramon R.
N1 - Funding Information: The authors have no conflicts of interest to disclose for the submitted work. Outside the submitted work Dr. Ramon R. Gorter and Dr. R. Bakx received a (governmental) ZonMw grant for research in the field of complex appendicitis in the pediatric population. Grant Number: 80-85009-98-2007. Publisher Copyright: © 2022 The Authors
PY - 2023/2
Y1 - 2023/2
N2 - This study systematically reviewed the literature to investigate the value of secondary surgery for children with a high-risk neuroendocrine tumor (NET) of appendix. A systematic search was performed in PubMed, Embase and Web of Science. All randomized controlled trials, cohort studies, and case series reporting on the management and outcomes of patients (<20 years) with a histopathologically proven NET of the appendix were eligible for inclusion. Two authors independently selected eligible articles, assessed risk of bias, and extracted data. The outcomes of patients with a high-risk NET treated with secondary surgery were compared to those treated without secondary surgery. Primary outcomes were recurrence rate and disease-free survival. The literature search yielded 667 articles, of which 29 were included. These studies reported on 1112 patients, of whom 145 (13%) had high-risk NET. Heterogeneity between studies was large and risk of bias was serious in 26 and moderate in three studies. Secondary surgery after primary appendectomy was performed in 64 of 145 patients (44%). Length of follow-up ranged between 0 and 612 months. In both treatment groups no recurrences were reported, and thus disease-free survival was 100%. Based on current literature, the value of secondary surgery for pediatric high-risk NET of the appendix may be questioned. However, evidence is scarce, of low-quality, and heterogeneity between studies is large. Large international studies with adequate follow-up are needed to generate high-quality evidence on this topic.
AB - This study systematically reviewed the literature to investigate the value of secondary surgery for children with a high-risk neuroendocrine tumor (NET) of appendix. A systematic search was performed in PubMed, Embase and Web of Science. All randomized controlled trials, cohort studies, and case series reporting on the management and outcomes of patients (<20 years) with a histopathologically proven NET of the appendix were eligible for inclusion. Two authors independently selected eligible articles, assessed risk of bias, and extracted data. The outcomes of patients with a high-risk NET treated with secondary surgery were compared to those treated without secondary surgery. Primary outcomes were recurrence rate and disease-free survival. The literature search yielded 667 articles, of which 29 were included. These studies reported on 1112 patients, of whom 145 (13%) had high-risk NET. Heterogeneity between studies was large and risk of bias was serious in 26 and moderate in three studies. Secondary surgery after primary appendectomy was performed in 64 of 145 patients (44%). Length of follow-up ranged between 0 and 612 months. In both treatment groups no recurrences were reported, and thus disease-free survival was 100%. Based on current literature, the value of secondary surgery for pediatric high-risk NET of the appendix may be questioned. However, evidence is scarce, of low-quality, and heterogeneity between studies is large. Large international studies with adequate follow-up are needed to generate high-quality evidence on this topic.
KW - Appendix
KW - Children
KW - Neuro-endocrine tumor
KW - Surgical treatment
UR - http://www.scopus.com/inward/record.url?scp=85141959202&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.ejso.2022.10.021
DO - https://doi.org/10.1016/j.ejso.2022.10.021
M3 - Review article
C2 - 36372617
SN - 0748-7983
VL - 49
SP - 329
EP - 338
JO - European Journal of Surgical Oncology
JF - European Journal of Surgical Oncology
IS - 2
ER -