Pancreatoblastoma and solid and cystic papillary tumor: two tumors related to pancreatic ontogeny.

ABSTRACT

Two tumors of the pancreas are related to pancreatic ontogeny. The pancreatoblastoma is a tumor of children, more commonly boys than girls and progresses with a slow, sluggishly malignant course. Surgery successfully controls the disease in about half the cases. Morphologic and immunohistochemical studies reveal undifferentiated areas, ductular areas, acinar areas, and occasionally neuroendocrine differentiation. Very characteristic is the presence of nodules of squamous epithelium. These features reflect the potencies of the pancreatic anlage somewhat earlier than the fourteenth week of development. The solid and cystic papillary epithelial tumor of the pancreas is a tumor of adolescent and young adult females, often non-Caucasian. Surgical excision is often successful, but deaths from local persistence and metastases have been described. These tumors have features of ductulo-acinar neoplasms of uncommitted pancreatic primordia exceptionally with neuroendocrine differentiation.