RARE PANCREATIC TUMOURS IN CHILDREN (OTHER THAN NESIDIOBLASTOSIS)

Background: The rarity of pancreatic tumours other than nesidioblastosis in children is such that the experience of any one surgeon or institution is small. As a consequence, there is limited information on the appropriate management and outcome of these tumours. For this reason a review was conducted of the experience of a large paediatric surgical institution. Methods: During a 23-year period, six patients with pancreatic tumours other than nesidioblastosis were treated at the Royal Children's Hospital, Melbourne. Results: Despite the varied modes of presentation and surgery undertaken, all six patients survived with surgery alone, with follow-up from 1 to 18 years. Two of the tumours were malignant; the remaining four being islet cell adenomata. No form of adjuvant treatment (chemotherapy or radiotherapy) was used. Conclusion: These rare paediatric tumours appear to have a good prognosis, even when malignant, and respond well to radical surgical ablation. Adjuvant therapy appears to be unnecessary.