Pancreatoblastoma.

ABSTRACT

Pancreatoblastoma is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non endocrine tumors. Just over 50 cases have been reported in the literature till 1997. A 10 year-old male child presented with mass abdomen of 9 months duration. USG and CT abdomen showed a large mixed density lobulated mass in abdomen anterior to, and displacing down the portal vein and splenic veins. Exploratory laparotomy revealed a large mass, arising from body and tail of pancreas, not infiltrating the adjacent structures. Complete excision of the mass along with removal of body and tail of pancreas was done. The histopathological examination revealed pancreatoblastoma. He is well without any evidence of disease 6 months after the operation. To the best of our knowledge this is the first case of pancreatoblastoma with a large size (25 x 20 x 15 cm,) weight 2.5 kg, which was localized and where complete excision was possible. The largest size reported in the literature reviews has been around 15 cm. The literature review confirms that an aggressive surgical attack on resectable pancreatoblastoma may often be curative. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to small number of patients treated as yet.