Pancreatoblastoma

Pancreatoblastomas are rare paediatric tumours of the pancreas. However, they are the most common pancreatic neoplasm of childhood and are often associated with a raised alpha-fetoprotein.

Epidemiology

There is slight male predilection. Usually occurs in the first decade of life with a mean age of 4.5 years ³.

Pathology

Pancreatoblastomas are solid tumours, flesh-coloured on gross pathology. They can occur in any part of the pancreas ⁴. 

Metastatic lesions in liver and lymphadenopathy have been reported in 35% of cases ³.

Associations

• Beckwith-Wiedemann syndrome

Radiographic features

Ultrasound

They are usually large, well defined, heterogeneous mass with solid and multilocular cystic areas that contain hyperechoic septae. Dilatation of biliary duct is not common as the tumours themselves are soft, though they are frequently large at presentation ³.

CT

• usually relatively well defined and heterogeneous due to solid and multilocular cystic components with enhancing septae
• may demonstrate fine calcifications, similar to those seen in neuroblastoma
• most often occurs in the head of the pancreas and tend to be large and solitary, though dilatation of bile duct is uncommon
• hepatic metastases: hypodense
• less frequently, invasion of adjacent structures and the biliary tree may be noted

MRI

• T1: low-intermediate signal intensity
• T2: heterogenous hyperintense

Treatment and prognosis

Has a better prognosis than adult pancreatic adenocarcinoma.

Differential diagnosis

• Wilms tumour
• neuroblastoma