Solid pseudopapillary neoplasms of the pancreas: a multi-institutional study of 21 patients.

Background: Solid pseudopapillary neoplasms of the pancreas (SPN) account for less than 1% of all pancreatic tumors. The goal of this study was to better understand the nature of these rare tumors through analysis of patients' clinical presentations and outcomes following surgical resection.

Methods: A multi-institutional retrospective review was conducted of all patients who underwent surgical resection from 1994 to 2008.

Results: Twenty-one patients were identified with SPN. Twenty patients were female. Median age at presentation was 34 y. The most common presenting symptom was abdominal pain (67%). All patients underwent resection: distal pancreatectomy (9), pancreaticoduodenectomy (5), central pancreatectomy (6), and laparoscopic excision/enucleation (1). A R(0) resection was obtained in all patients. Median tumor size was 5.5 cm. AJCC stages were stage I (18), stage II (1), stage III (2), and stage IV (0). Postsurgical complications occurred in 52% of patients, with pancreatic fistulae being the most common (29%). The median follow-up time was 55 mo. All patients remain alive without evidence of recurrence.

Conclusion: Solid pseudopapillary neoplasms of the pancreas are atypical pancreatic tumors. SPN usually occur in young women who present with abdominal pain. Oncologic outcomes in patients who undergo surgical resection are excellent.