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Appendiceal Neuroendocrine Tumors in Children and Adolescents

Jelena Roganovic1, Luisa Santoro2, Calogero Virgone3

  • 1Department of Pediatric Hematology and Oncology, Children's Hospital Zagreb, Zagreb, Croatia; Faculty of Biomedicine and Drug Development, University of Rijeka, Rijeka, Croatia. jelena.roganovic02@gmail.com; jelena.roganovic@kdbz.hr.

Acta Medica Academica
|March 31, 2026
PubMed

Insights

Pediatric appendiceal neuroendocrine tumors (aNETs) are rare and usually indolent. Most cases are cured with simple appendectomy, emphasizing a risk-adapted, pediatric-specific approach to management.

Area of Science:

  • Pediatric Oncology
  • Gastrointestinal Surgery
  • Neuroendocrine Tumors

Background:

  • Appendiceal neuroendocrine tumors (aNETs) are rare in children and adolescents.
  • Pediatric aNETs typically have an indolent course with low metastatic potential.
  • Biological and prognostic features differ significantly from adult aNETs, precluding direct application of adult guidelines.

Purpose of the Study:

  • Synthesize current evidence on pediatric aNETs.
  • Detail diagnosis, histopathology, clinical features, management, and follow-up.
  • Highlight differences between pediatric and adult aNETs.

Main Methods:

  • Mini-review of current literature.
  • Focused on epidemiology, clinical presentation, diagnostic workup, pathology, surgical management, and follow-up.
  • Included pediatric appendiceal neuroendocrine tumors (aNETs).

Main Results:

  • Contemporary evidence supports a de-escalated, risk-adapted management strategy.
  • Simple appendectomy is curative for the majority of pediatric aNET cases.
  • Multidisciplinary Team (MDT) discussion is crucial for complex or borderline cases.

Conclusions:

  • Early recognition and accurate histopathologic evaluation are vital.
  • Tailored surgical management optimizes outcomes for pediatric patients.
  • Pediatric-specific clinical decision-making is necessary due to differences from adult aNETs.
Abstract

Keywords:
AppendixChildren and AdolescentsNeoplasmsNeuroendocrine

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