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Bening behavior of pediatric appendiceal NETs: is there a need for additional surgery?
Duygu Gürel 1, Songül Arabul Aydoğdu 1, Şeymanur Atcı 1, Doğakan Yiğit 1, Nazlı Ecem Kore 2, Mehmet Oğuz Erguvan 2, Oktav Bosnalı 1
1 Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Pediatric Surgery and Pediatric Urology, İstanbul
2 University of Health Sciences Prof. Dr. Cemil Taşcıoğlu City Hospital Department of Pathology
Introduction: Neuroendocrine tumors of the appendix (aNET) are rare in children even though they are considered one of the most common gastrointestinal pediatric neoplasms. Due to their rarity in pediatric population, there is no consensus on treatment and follow-up. We aimed to discuss the management of two patients who were incidentally diagnosed with aNET following histopathological examination after appendectomy.
Case 1: A 16-year-old male presented with 48 hours of right lower quadrant pain. Laboratory tests and abdominal computed tomography (CT) confirmed acute appendicitis, and laparoscopic appendectomy was performed. Histopathological examination revealed a 1 mm, WHO grade 1 aNET in the distal appendix, invading the submucosa. No lymphovascular or mesoappendiceal invasion was noted, and resection margins were clear. No further intervention was required. The patient remains recurrence-free after 8 months of follow-up.
Case 2: A 12-year-old female presented with generalized abdominal pain and signs of acute abdomen. Abdominal CT revealed free air and perihepatic fluid. Due to intra-abdominal adhesions and extensive abscess formation, the procedure was converted to laparotomy, which revealed a perforated appendix. Histolopathological examination revealed a 1,2x0.6×0.7 cm, WHO grade 2 aNET infiltrating into the subserosa, without lymphovascular or perineural invasion. Margins were clear. A PET-CT scan was performed for metastatic evaluation. No additional surgery was planned, and she remains well at 9-month follow-up.
Conclusion: ANETs in children have an excellent prognosis and rarely require surgery beyond appendectomy. Our findings support the benign nature of this tumor and demonstrate that appendectomy alone is sufficient treatment in most pediatric cases; surgery remains the mainstay of management, with tumor size being the most critical factor in determining the extent of surgical intervention. Given the lack of international consensus and large-scale data, collaborative studies and pediatric-specific guidelines are warranted.