Solid pseudo-papillary tumor (SPPT) is a rare cystic tumor of
the pancreas (1-3% of exocrine tumors of the pancreas) which
shows an “enigmatic” behavior on the clinical and molecular pattern.
A retrospective analysis of the citological studies and resected
specimens of pancreatic cystic tumors from May 1996 to February
2010 was carried out. Three cases of SPPT were found,
which are the objective of this study. The diagnosis was established
upon occasional finding in the abdominal CT, in spite of sizing
between 3 and 6 cm of diameter. In the three cases the preoperative
diagnosis was confirmed by citology and specific
immunohistochemical staining. Cases 2 and 3 showed strong immunoreactivity
for Beta-Catenina and E-Cadherina staining. Radical
resection (R0) was carried out in the three cases. A young
male –21 years of age (case 1)- who had duodenal infiltration and
two lymph nodes metastases died of hepatic and peritoneal recurrence
20 months following surgery. The other two cases are free
of disease. The current review of the literature reports roughly
800 cases since the first report in 1959, and shows the enigmatic
character of this tumor regarding the cellular origin, molecular
pathways, prognostic factors and clinical behavior