Appendiceal Neuroendocrine Tumors in Children and Adolescents

OBJECTIVE: To synthesize current evidence on the diagnosis, histopathological evaluation, clinical features, management, and follow-up of appendiceal neuroendocrine tumors (aNETs) in children and adolescents, and to outline key differences from their adult counterparts. BACKGROUND: Pediatric aNETs are rare gastrointestinal neoplasms that typically exhibit an indolent clinical course with minimal risk of recurrence or metastasis. Their biological and prognostic features differ from those in adults, limiting the applicability of adult-derived guidelines in children. METHODS: A mini review of the current literature was conducted, focusing on epidemiology, clinical presentation, diagnostic workup, key pathological features, surgical management, and follow- up strategies for pediatric aNETs. DISCUSSION: Contemporary evidence supports a de-escalated, risk-adapted approach to management, with simple appendectomy being curative in most cases. Multidisciplinary Team (MDT) discussion remains critical for atypical or borderline cases requiring individualized decision-making. Differences from adult aNETs highlight the need for pediatric-specific clinical decision-making. CONCLUSION: Early recognition, accurate histopathologic evaluation, and tailored surgical management are essential to optimize outcomes for children and adolescents with aNETs.