A National Long-Term Study of Neuroendocrine Tumors of the Appendix in Children: Are We Too Aggressive?

Details

Serval ID
serval:BIB_4EA027D130BC
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
A National Long-Term Study of Neuroendocrine Tumors of the Appendix in Children: Are We Too Aggressive?
Journal
European journal of pediatric surgery
Author(s)
Sommer C., Gumy Pause F., Diezi M., Rougemont A.L., Wildhaber B.E.
ISSN
1439-359X (Electronic)
ISSN-L
0939-7248
Publication state
Published
Issued date
10/2019
Peer-reviewed
Oui
Volume
29
Number
5
Pages
449-457
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Abstract
Pediatric neuroendocrine tumors (NETs) of the appendix are mostly detected incidentally after appendectomy for acute appendicitis. NET management is a matter of controversy. In this national, multicenter study, we aimed to establish guidelines based on our results and the literature.
Medical records of children (0-16 years) with NET of the appendix, treated in Switzerland (1991-2012), were reviewed.
Forty cases (28 girls) were analyzed. Median age at diagnosis was 12.7 years (interquartile range [IQR]: 4.0). Tumor size was 0.1-24 mm (median: 0.6, IQR: 0.6). Four patients (10%) underwent additional surgery because of either tumor size > 15 mm (1/4), extension to the mesoappendix (1/4), or incomplete resection (2/4). Three patients with a tumor of ≥ 20 mm had no additional surgery. No patient had lymph node metastases. All patients were in complete remission at the last follow-up (median: 3.0 years, IQR: 10.9).
We conclude from this study and from an extensive review of the literature that two criteria may point to the need for additional surgery, i.e., the possibility of regional lymph node involvement: tumor size > 20 mm and incomplete surgical resection margins. Childhood NET of the appendix has an excellent prognosis.
Keywords
Adolescent, Appendiceal Neoplasms/pathology, Appendiceal Neoplasms/surgery, Appendicitis/complications, Appendicitis/surgery, Child, Child, Preschool, Humans, Lymphatic Metastasis/pathology, Male, Margins of Excision, Neoplasm Invasiveness, Neuroendocrine Tumors/pathology, Neuroendocrine Tumors/surgery, Retrospective Studies
Pubmed
Web of science
Create date
08/10/2018 8:12
Last modification date
05/04/2020 5:20
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