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Congenital Pancreatoblastoma: Report of an Atypical Case and Review of the Literature JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY Chisholm, K. M., Hsu, C. H., Kim, M. J., Rangaswami, A., Hazard, F. K. 2012; 34 (4): 310-315

Abstract

Pancreatoblastoma is a rare malignant tumor of the pancreas mostly diagnosed in childhood. The clinical presentation and outcome of infantile and congenital pancreatoblastoma have not been clearly elucidated. This report describes our recent institutional experience with an unusual case of congenital pancreatoblastoma. Review of the scientific literature identifies approximately 200 cases of pancreatoblastoma. We describe the 9 infantile (aged 3 mo and younger) and 4 congenital cases previously reported and summarize their clinical presentation and outcome. We also define the close association of infantile/congenital pancreatoblastoma and Beckwith-Wiedemann syndrome (50%) versus all affected age groups (4.5%).

View details for DOI 10.1097/MPH.0b013e318239f4f6

View details for PubMedID 22278199