摘要
A case of pancreatoblastoma (PB) in a 2 month-oldmale infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical exa mination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, a nd enlargement of the left testis. Imaging with ultrasound and computed tomograp hy scan showed a well-defined, heterogeneous, and grossly cystic mass arising f rom the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient’s recovery was uneventful, and AFP returned to normal values after sur gery. The child has been disease-free for 5 years, and his serum AFP remained w ithin normal values. Six other examples of this association, PB, and Beckwith-W iedemann syndrome are recorded in the literature. The risk of developing tumor i n this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith -Wiedemann syndrome was suggested to be a favorable biological marker for survi val in children who have intraabdominal tumors.
A case of pancreatoblastoma (PB) in a 2 month-oldmale infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical exa mination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, a nd enlargement of the left testis. Imaging with ultrasound and computed tomograp hy scan showed a well-defined, heterogeneous, and grossly cystic mass arising f rom the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after sur gery. The child has been disease-free for 5 years, and his serum AFP remained w ithin normal values. Six other examples of this association, PB, and Beckwith-W iedemann syndrome are recorded in the literature. The risk of developing tumor i n this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith -Wiedemann syndrome was suggested to be a favorable biological marker for survi val in children who have intraabdominal tumors.
