Histopathologic subtypes and clinical evaluation of appendiceal neuroendocrine tumor in children: Single center experience

INTRODUCTION: Appendiceal neuroendocrine tumors (A-NETs) are rare neoplasms that are usually incidentally diagnosed following appendectomy. This study aimed to review our experiences with A-NETs in children.
METHODS: This retrospective study analyzed 3800 children who underwent urgent appendectomy for clinical suspicion of appendicitis at our hospital between 2010 and 2023. Of the appendectomy specimens, 14 were diagnosed as A-NET.
RESULTS: The study included 14 patients with a mean age of 14.14 years (range 2–17), consisting of 9 (64.2%) males and 5 (35.7%) females. The mean tumor diameter was 5.5 mm (range 2–17 mm), and all tumors were located in the distal appendix. Tumor invasion was observed in the submucosa in one case, muscularis propria in seven cases, subserosa in two cases, serosa in one case, and mesoappendix in three cases. In all cases, resection margins were tumor-free. At the last follow-up (mean follow-up 49.7 months), all patients were alive and without evidence of disease. No recurrence or metastasis was observed. The present study identified 11 tumors with the classic insular growth pattern of solid islands, 2 with a tubular pattern, and 1 with a mixed classic and tubular pattern.
DISCUSSION AND CONCLUSION: A-NETs generally have a favorable prognosis in pediatric patients. Hemicolectomy is not recommended for patients with completely resected tumors.