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Appendectomy vs Right Hemicolectomy for Pediatric Neuroendocrine Tumor of the Appendix in a National Cohort: A Call
Swathi R Raikot1, Donald Dean Potter1, Courtney N Day2
1From the Division of Pediatric Surgery, Departments of Surgery (Raikot, Potter, Gudmundsdottir, Polites), Mayo Clinic, Rochester, MN.
Insights
Appendectomy alone is sufficient for treating pediatric neuroendocrine tumors (NET) of the appendix, even with positive margins. This approach ensures excellent survival rates in children, challenging traditional adult guidelines.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Gastrointestinal Oncology
Background:
- Children with appendiceal neuroendocrine tumors (NET) are often treated with right hemicolectomy (RHC) based on adult guidelines.
- Appendectomy alone is recommended for pediatric cases, but its safety with positive margins is not well-established.
Purpose of the Study:
- To evaluate the association between tumor characteristics and survival in a national cohort of pediatric patients with appendiceal NET.
- To determine if appendectomy alone is a safe and effective treatment, even in the presence of high-risk features like positive margins.
Main Methods:
- A national cohort of 1,339 patients aged 18 years or younger with appendiceal NET was identified (2004-2022).
- Patient data from the National Cancer Database were analyzed, comparing outcomes between appendectomy and RHC.
- Five-year survival rates were assessed for appendectomy patients with and without high-risk features (size, margin, lymph node status).
Main Results:
- Appendectomy was performed in 86% of patients (1,156) versus RHC in 14% (183).
- Patients undergoing RHC had larger tumors and more lymphovascular invasion, but margin status showed no difference.
- Overall 5- and 10-year survival rates were excellent (99.9% and 99.4%), including for those treated with appendectomy alone (99.9% and 99.2%).
Conclusions:
- Appendectomy is confirmed as the definitive management for pediatric appendiceal NET.
- Further resection (RHC) does not improve survival outcomes, even with positive margins on appendectomy specimens.
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 18 years of age or younger 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 to 17) years. Patients who underwent RHC had larger tumors (24% vs 4%, greater than 2 cm, p < 0.001), more lymphovascular invasion (31% vs 10%, p < 0.001), and were more likely to undergo LN assessment (82% vs 10%, p < 0.001) with a higher LN positivity rate (31% vs 16%, p = 0.006). 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%).
Conclusions:
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.
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