Appendiceal neuroendocrine tumor in a pediatric patient – A case report

Abstract

Appendiceal neuroendocrine tumors (NETs) are the most common appendiceal neoplasms but remain rare in the pediatric population. They often present with clinical features mimicking acute appendicitis and are therefore usually diagnosed incidentally on histopathological examination after appendectomy. Prompt identification is crucial, as tumor characteristics determine management and prognosis. We report a case of a 13-year-old girl presenting with clinical and radiological signs of acute appendicitis due to fecalith obstruction and a right ovary cyst. Laparoscopic appendectomy and ovarian cystectomy were performed without intraoperative suspicion of malignancy. Histopathological analysis revealed a well-differentiated neuroendocrine tumor measuring 1.5 cm localized near the tip of the appendix. The lesion infiltrated all the layers of the appendix wall and penetrated the serosa, without lymphovascular invasion or mesoappendiceal extension. Immunohistochemical staining confirmed the diagnosis. Multidisciplinary tumor board decision guided further management and the patient remains disease-free at twelve-month follow-up. Outcome was promising with no recurrence, metastasis, complications, or need for additional therapy observed during follow-up. The discussion section emphasizes the critical role of histopathological analysis of appendectomy specimens and gives further recommendations for efficient management of appendiceal NETs in children, depending on tumor size and histological risk features. Appendectomy alone is usually sufficient for tumors less than 2 cm without invasion. This case underscores the critical role of histopathological vigilance and multidisciplinary care in pediatric surgical oncology.