DEFINITION
Pancreatoblastoma is a primary malignant tumor of the pancreas consisting of an epithelial component showing acinar differentiation, nests of squamoid cells (or squamoid corpuscles), and occasional endocrine cells. There may be a mesenchymal component in some tumors. Although the tumor has a predilection for children, it has also been reported in adults. Synonyms for this tumor include pancreaticoblastoma and carcinoma of the pancreas, infantile type
GROSS PATHOLOGY
Pancreatoblastoma presents as a solitary mass that can arise in any location in the pancreas. The tumor tends to be relatively large, ranging from 7 to 18 cm, and on cross section it has been described as being tan-yellow with incomplete lobulations. The consistency may vary from soft and fleshy to focally fibrous, and there may be areas of necrosis and hemorrhage. Cystic change may be present, and on rare occasion a gritty texture has been described due to abundant calcification.
MICROSCOPIC PATHOLOGY
Pancreatoblastoma may have a partial fibrous pseudocapsule, but there is often invasion into adjacent pancreas, duodenum, or soft tissues. The first case of pancreatoblastoma was illustrated in 1959 by Frantz , and it was the same case reported by Becker in 1957 as the first pancreatic tumor in a child (a 15-month-old boy) treated by pancreaticoduodenectomy.
Pancreatoblastoma is a primary malignant tumor of the pancreas consisting of an epithelial component showing acinar differentiation, nests of squamoid cells (or squamoid corpuscles), and occasional endocrine cells. There may be a mesenchymal component in some tumors. Although the tumor has a predilection for children, it has also been reported in adults. Synonyms for this tumor include pancreaticoblastoma and carcinoma of the pancreas, infantile type
GROSS PATHOLOGY
Pancreatoblastoma presents as a solitary mass that can arise in any location in the pancreas. The tumor tends to be relatively large, ranging from 7 to 18 cm, and on cross section it has been described as being tan-yellow with incomplete lobulations. The consistency may vary from soft and fleshy to focally fibrous, and there may be areas of necrosis and hemorrhage. Cystic change may be present, and on rare occasion a gritty texture has been described due to abundant calcification.
MICROSCOPIC PATHOLOGY
Pancreatoblastoma may have a partial fibrous pseudocapsule, but there is often invasion into adjacent pancreas, duodenum, or soft tissues. The first case of pancreatoblastoma was illustrated in 1959 by Frantz , and it was the same case reported by Becker in 1957 as the first pancreatic tumor in a child (a 15-month-old boy) treated by pancreaticoduodenectomy.
