Case report: neuroendocrine tumour causing torsion of the appendix in an adolescent—a rare and unexpected twist

Background: Neuroendocrine tumours of the appendix are rare in the paediatric population, with an incidence of 1 in 10,000. They tend to be diagnosed incidentally following appendicectomy for suspected or confirmed appendicitis.

Case Description: A 14-year-old female who presented to the emergency department with a 2-day history of migratory right lower quadrant pain. She had no significant medical or surgical history. She was tender in the right iliac fossa on examination with no classical signs of peritonitis. Mildly raised inflammatory markers along with a non-diagnostic ultrasound lead to the decision to proceed to diagnostic laparoscopy. A torted, injected appendix was identified and removed intraoperatively. The patient had an uncomplicated recovery and was discharged on post operative day one. Histopathology demonstrated a well differentiated grade 2 neuroendocrine tumour with clear margins. The case was discussed at an institutional multidisciplinary team (MDT) with the decision for ongoing surveillance.

Conclusions: We describe the first case of a neuroendocrine tumour as the lead point for a torted appendix. Clinicians should be cognisant of the increasing incidence of neuroendocrine tumours and perform adequate resections in any cases where there are unusual findings. Currently management is based on adult management guidelines and these may be too aggressive for paediatric populations. The development of paediatric guidelines should be considered.