Diagnosis, treatment and outcome of pancreatoblastoma.
BACKGROUND
Pancreatoblastoma is a rare tumour mainly presenting in childhood but also in adults.


OBJECTIVE
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.
Dhebri
A R
AR
Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK.
Connor
S
S
Campbell
F
F
Ghaneh
P
P
Sutton
R
R
Neoptolemos
J P
JP
D002648:Child; D005260:Female; D006801:Humans; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D011379:Prognosis; D016019:Survival Analysis; D016896:Treatment Outcome