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BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is a distinctive pancreatic neoplasm with low metastatic potential. This study examines clinical differences and prognosis between male and female patients. METHODS: The medical records of 34 consecutive patients with pancreatic solid pseudopapillary neoplasms between 1990 and 2006 were reviewed. Whenever feasible, organ-preserving operation was performed. Statistical analysis was performed using chi-square and Student t test. RESULTS: There were 27 women (79%) and seven men (21%) with median age of 23 years. Mean diameter of the tumor was 7 cm . Tumor size tended to be smaller in patients treated in more recent years. Conservative surgery was possible in 11 patients including spleen-preserving distal pancreatectomy in 3, central pancreatectomy in 5, and enucleation in 3 patients. Median hospital stay was 11 days, morbidity rate was 62%, including 17 patients with grade A pancreatic fistula, and there was no operative mortality. Mean follow-up time was 84 months. Tumor recurred in 2 patients (6%). Overall late morbidity rate was 12%. At the time of diagnosis, age was (x +/- SD) higher among male patients (25 +/- 2 years vs 37 +/- 7 years; P <.05) with no difference in tumor size. The neoplasms were more aggressive in male patients; therefore, conservative surgery was less likely. There was no correlation between tumor aggressiveness and age of the patient or size of tumor. CONCLUSION: This is the first single center study to demonstrate that solid pseudopapillary neoplasms in male patients have distinct patterns of onset and aggressiveness when compared with female patients. Although valid prognostic criteria are still lacking, it appears that male patients may be best treated by more radical operation and should be observed more closely during follow-up.