Pancreatic tumours in children: diagnosis, treatment and outcome.

PURPOSE: Pancreatic tumours in children are exceedingly rare and as a result constitute a diagnostic and therapeutic challenge to paediatric surgeons. We reviewed our experience with these rare entities.

METHODS: Retrospective single institution study on all paediatric pancreatic tumours over a period of 38 years (1973-2011) and literature review. We recorded data relating to the clinical features at presentation, diagnostic evaluation, treatment and outcome.

RESULTS: Fourteen patients were identified (eight male). The most common symptoms at presentation were abdominal pain, anorexia and vomiting. Two cases were discovered incidentally. There were 12 primary and 2 metastatic tumours. Tumour types were solid pseudopapillary neoplasm (n = 6), insulinoma (n = 3), pancreatoblastoma (n = 1), congenital pancreatic cyst (n = 1), Burkitt lymphoma of the pancreas (n = 1) and metastatic lesions of other primary tumours (n = 2). Twelve were treated with surgical resection including 2 laparoscopically. Post-surgical complications included acute pancreatitis (n = 2) and pancreatic pseudocyst (n = 2). There was one death from a metastatic tumour and treatment is ongoing in one patient. The remainder has survived.

CONCLUSION: Paediatric pancreatic tumours are rare entities and are usually benign. Clinical symptoms are often non-specific and presentation may be late due to tumour inactivity in case of endocrine neoplasms. For most tumours, surgical resection is the optimal treatment which may be successfully performed laparoscopically when the lesion is in the body or tail of the pancreas. Long-term outcome is generally good.