PAPILLARY CYSTIC AND SOLID TUMORS OF THE PANCREAS - A PANCREATIC EMBRYONIC TUMOR - STUDIES OF 3 CASES AND CUMULATIVE REVIEW OF THE WORLDS LITERATURE

Background. The papillary cystic and solid tumor of the pancreas is ra re. It occurs predominantly in young women, and most present a benign behavior. The pathogenesis of this turner has attracted a number of in vestigations but remains unclear. Methods. We present three patients w ith this tumor and a review of 289 others from the world's literature, a total of 292 cases. On the basis of the analyses of the clinical an d pathologic features from the reported cases, the pathogenesis of thi s unusual tumor has been further explored. Results. Ninety percent of the patients were female, with a mean age of 23.9 years. The tumors we re usually quite large with a mean diameter of 10.3 cm. Ninety-two per cent of these tumors were totally or partially cystic. Rupture of the capsule resulted in hemoperitoneum in eight cases, five of which were without any identifiable cause. Forty-three tumors (14.7%) have been r ecognized as malignant. The overall prognosis has been excellent and a n aggressive approach to resection is indicated. Conclusions. The resu lts of immunohistochemical staining and electromicroscopy were rather diverse, but most, including the current cases, support the hypothesis that the tumor originates from pleuripotential embryonic stem cells. Thus the term pancreatic embryonic tumors seems preferable to papillar y cystic and solid tumor of the pancreas to delineate the origin of th e tumor and to reflect some of its biologic characteristics.