Surgical Management of Paediatric Appendiceal Neuroendocrine Tumors: A 26-Year Danish Nationwide Retrospective Cohort Study.

BACKGROUND

Appendiceal neuroendocrine tumors (aNET) are incidental findings after appendectomy. Paediatric guidelines on surgical management are lacking, and the need for further surgery is debated. This retrospective cohort study aimed to describe incidence, patient and tumor characteristics, surgical management and outcomes, and assess criteria for right-sided-hemicolectomy.

METHODS

We conducted a nationwide retrospective cohort study including all children (≤ 18 years) diagnosed with aNET from 1995 to 2020. A total of 205 children were identified from the Danish National Pathology Registry.

MAIN FINDINGS: The annual incidence of aNET was 6.37 per million children. Median age at diagnosis was 15 years (range 4-18) with a female-to-male ratio of 1.85:1. Identified histopathological risk factors included tumor size >20 mm, tumor location at the base, positive resection margins, mesoappendiceal invasion >3 mm, lymphovascular invasion, Ki-67 index ≥3 %, and tumor stage T3/T4. Median tumor size was 8 mm - seven patients had tumors >20 mm. Positive resection margins were found in 10 patients. Forty-four patients had mesoappendiceal invasion. Seventeen patients had a grade 2 tumor (Ki-67 index 3-20 %). Tumor stage ≥ T3 was given to 42 patients. Secondary surgery was performed in 44 children (21.5 %), with no residual tumor identified. Lymph node metastasis was found in three patients. No recurrence or aNET-related mortality occurred.

CONCLUSION

The absence of recurrence or aNET-related mortality supports the notion that aNET carries an excellent prognosis. The prognostic value of histopathological risk factors may be questionable. Paediatric-specific surgical guidelines are needed to prevent unnecessary surgeries and associated complications.