Background: Children continue to undergo right hemicolectomy (RHC) for neuroendocrine tumors (NET) of the appendix based on adult guidelines. Appendectomy alone is recommended for the pediatric population; however, there are no data on safety of this approach in the setting of positive margins. This study aimed to determine the association between tumor characteristics and survival in a national cohort, hypothesizing that survival would be excellent despite high-risk features including positive margins on appendectomy.
Study design: Patients aged ≤ 18 years with NET of the appendix were identified in the National Cancer Database from 2004 to 2022 using ICD-O-3 codes. Characteristics of patients who underwent definitive appendectomy vs RHC were compared using Chi-square tests. Five-year survival was determined for appendectomy patients with and without high-risk size, margin, and lymph node (LN) features.
Results: Of 1,339 patients, 1,156 (86%) underwent appendectomy and 183 (14%) RHC. Median age in both groups was 15 (13, 17) years. Patients who underwent RHC had larger tumors (24% vs 4% >2 cm, p<.001), more lymphovascular invasion (31% vs 10%, p<.001), and were more likely to undergo LN assessment (82% vs 10%, p<.001) with a higher LN positivity rate (31% vs 16%, p=.006). There was no difference in margins status (p=0.76). The 5- and 10-year survival was excellent regardless of tumor characteristics for patients overall (99.9% and 99.4%, respectively) and for those who underwent definitive appendectomy (99.9% and 99.2%).
Conclusion: These data further support appendectomy as definitive management for children with appendiceal NET. Additional resection does not confer a survival benefit even in the setting of positive margins.
Keywords: Appendectomy; Carcinoid; Neuroendocrine tumor; Pediatric; Right hemicolectomy.
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