Eur J Pediatr Surg
DOI: 10.1055/s-0038-1667032
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Christelle Sommer
1  Department of Pediatric Surgery, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
,
Fabienne Gumy Pause
2  Department of Pediatric Hemato-oncology, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
,
Manuel Diezi
3  Unit of Pediatric Hemato-oncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Canton of Vaud, Switzerland
,
Anne-Laure Rougemont
4  Division of Clinical Pathology, Hopitaux Universitaires de Geneve, Geneva University Hospitals, Geneva, Switzerland
,
Barbara E. Wildhaber
1  Department of Pediatric Surgery, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
› Author Affiliations
Further Information

Publication History

19 April 2018

04 June 2018

Publication Date:
01 August 2018 (eFirst)

Abstract

Background 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.

Methods Medical records of children (0–16 years) with NET of the appendix, treated in Switzerland (1991–2012), were reviewed.

Results 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).

Conclusion 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.