Diagnosis, treatment and outcome of pancreatoblastoma

Background: Pancreatoblastoma is a rare tumour mainly presenting in childhood but also in adults.

Objectives: The aim was to determine the clinical course of pancreatoblastoma by an analysis of reported cases.

Methods: Patients with pancreatoblastoma were identified from Medline and combined with patients identified from the Royal Liverpool University Hospital.

Results: There were 153 patients with a median (range) age at presentation of 5 (0-68) years and a male:female ratio of 1.14:1. The most frequent site was the head of pancreas (48/123, 39%). The median and 5-year (95% CI) survival rates were 48 months and 50% (37-62%) respectively. At presentation there were 17 (17%) out of 101 patients with metastases, the liver being the commonest site (15/17, 88%). On univariate analysis, factors associated with a worse prognosis were synchronous (p = 0.05) or metachronous metastases (p < 0.001), non-resectable disease at presentation (p < 0.001) and age > 16 years at time of presentation (p = 0.02). On multivariate analysis, resection (p = 0.006) and metastases post-resection (p = 0.001) but not local recurrence influenced survival.

Conclusions: Pancreatoblastoma is one of the pancreatic tumours with a relatively good prognosis. The treatment of choice is complete resection with long-term follow-up aiming to treat any early local recurrence or metastasis.