Infantile Extrapancreatic Pancreatoblastoma; Report of a rare infantile abdominal mass

Introduction:Pancreatoblastoma is an extremely rare tumor in children especially under 3 months of age.This tumor may arise from any portion of the pancreas but in more rare cases ectopicpancreas is the origin.Case presentation:We are reporting a 3 month old boy who was presented with an abdominal mass. Computedtomographies showed a huge lobulated mass anterior to the kidneys, with internalcalcification and enhancement after intravenous contrast media injection. He underwent acomplete surgical resection of the mass which was located in the transverse mesocolonwithout any connection with the pancreas. Pathologic studies specified that the disease waspancreatoblastoma. His parents refused any chemotherapeutic regimens, but continued postsurgicalfollow-ups.Conclusion:Pancreatoblastoma is a slow growing retroperitoneal tumor and usually is not symptomaticuntil it becomes quite large and lobulated. Most infantile cases were detected prenatally ornear birth. In the reported case it was occurred as an asymptomatic accidentally foundabdominal mass. Alfa fetoprotein might be useful for diagnosis, evaluation of the therapy andalso monitoring of postoperative recurrence in patients. The pathologic criteria ofpancreatoblastoma includes a calcified mass with solid and cystic elements, necrosis,presence of acinar cells, and a distinct organoid pattern containing lobular structures andsquamoid corpuscles in the region of pancreas.

کلیدواژه ها:

Extrapancreatic ، Pancreatoblastoma