Background: Pseudo-papillary Tumor (SPT) or Frantz tumor is a rare type of pancreatic tumor with an incidence of only 0.5-5% of all
pancreatic tumors. It was first described in 1959. SPT has a different behavior than pancreatic ductal adenocarcinoma (PDA)
regarding its gender predilection, presenting symptoms, metastatic potentiality and survival. Preoperative diagnosis of SPT is also
more difficult than PDA due to variety of findings, their presentation in imaging range from completely solid to completely cystic
tumors, and biopsy is less accurate in SPT diagnosis than PDA. Aim: to review and present National Cancer Institute (NCI), Cairo
University experience over the last 17 years in treating SPT, including diagnostic challenges and treatment outcome. Patients and
methods: This is a retrospective case series study of all cases of pancreatic pseudo-papillary tumor that were treated in NCI, in the
period between January 2003 and December 2019. Demographic data of the patients, their presenting symptoms, investigations
results, modality of treatment done and outcomes were collected from hospital records. Results: during this period 45 cases of SPT
were treated. Most of them were females (41/45). Body of pancreas was the most common site of the tumor (18/45). Only one case
had distant metastasis during initial presentation. Whipple procedure was done only in 12 cases. With follow up period ranged from
1-147 months, the 5 year disease free survival and overall survival were 86.4%, 92.1% respectively. Conclusion: SPT has a good
prognosis, as regarding DFS and OS even in large tumor size, also SPT has low metastatic potential compared to PDA.
Keywords: Pseudo-papillary tumor, pancreatic tumor, whipple