Neuroendocrine tumors of the appendix in children and adolescents

Background Neuroendocrine tumors (NET) of the appendix in children and adolescents are rare and mostly detected postoperatively by a histopathological examination. Since the malignant potential of these tumors remains unclear, therapeutic recommendations are not evidence based. The Society of Pediat...

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Published in:Pediatric blood & cancer 2013-01, Vol.60 (1), p.65-70
Main Authors: Boxberger, Nicole, Redlich, Antje, Böger, Christine, Leuschner, Ivo, von Schweinitz, Dietrich, Dralle, Henning, Vorwerk, Peter
Format: Article
Language:English
Subjects:
Adolescent
Adult
appendectomy
Appendiceal Neoplasms - pathology
Appendiceal Neoplasms - surgery
appendix
carcinoid
Child
Child, Preschool
children
Children & youth
Female
Follow-Up Studies
Hematology
hemicolectomy
Humans
Lymphatic Metastasis
Male
Medical Staff, Hospital
neuroendocrine tumor
Neuroendocrine Tumors - pathology
Neuroendocrine Tumors - surgery
Oncology
Pediatrics
Prospective Studies
ROC Curve
Tumors
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Summary:Background Neuroendocrine tumors (NET) of the appendix in children and adolescents are rare and mostly detected postoperatively by a histopathological examination. Since the malignant potential of these tumors remains unclear, therapeutic recommendations are not evidence based. The Society of Pediatric Oncology and Hematology (GPOH) has prospectively registered and followed children with appendical NET since 1997 (GPOH‐MET trial). The objective of this study was to critically evaluate the therapeutic recommendations for appendical NET in children. Procedure Clinical data of 237 children with appendical NET were prospectively analyzed. Results The mean age at presentation was 13.0 years, while the mean follow‐up time was 2.9 (0.0–12.8) years. The majority of tumors (70.9%) were located at the apex, and were smaller than or equal to 10 mm (72.7%). Tumor size was directly correlated with age. A second operation or primary lymph node sampling was done in 25.3% (N = 60) of the patients, and infiltration of the lymph nodes by tumor cells was found in nine of these patients (15%). All 237 patients are alive without tumor symptoms. Conclusions ROC analysis and subsequent calculations identified a tumor size of >15 mm as the optimal cut‐off point for the prediction of metastatic spread into the lymph system, with a sensitivity of 77.8% and a specificity of 66.7%. Therefore, secondary right hemicolectomy in completely removed appendical NET is recommended only in tumors >15 mm in size. For incompletely removed tumors ≤15 mm a local follow‐up resection with lymph node sampling is recommended. Pediatr Blood Cancer 2013; 60: 65–70. © 2012 Wiley Periodicals, Inc.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.24267