Background: Solid pseudopapillary neoplasms of the pancreas are rare malignant lesions of the pancreas that typically occur in young women. Large series from any one centre are notably absent in the literature. The aim of this study was to determine long-term outcomes of operative therapy. Methods: The records of all 14 patients diagnosed with pseudopapillary neoplasms of the pancreas over 17 years were reviewed. Results: Thirteen of the 14 patients were female and the mean age at diagnosis was 30 years. Solid pseudopapillary neoplasm was suspected in only half of these patients before operation. On computed tomography, ultrasonography and/or magnetic resonance imaging, three lesions were solid, three were largely cystic, and five had solid and cystic components. All 14 patients underwent surgical exploration and curative resections were possible in 13, including distal pancreatectomy in nine, pancreaticoduodenectomy in three and resection of a local intraperitoneal recurrence in one patient. After follow-up ranging from 3 months to 20 years, 12 patients were alive, including one who had undergone re-exploration and resection of local and subcutaneous recurrences 9 years previously. Conclusion: Solid pseudopapillary neoplasm of the pancreas should be considered in the differential diagnosis of any solid or partly cystic pancreatic mass in women aged less than 35 years. An attempt at en bloc resection without formal lymphadenectomy should be undertaken, including resection of synchronous or metachronous distant metastases.
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