Background: Solid pseudopapillary tumor of the pancreas (SPTP) is a rare disease with a low-grade malignant potential. In recent years, the incidence has been increasing. No consensus has been reached regarding diagnostic or therapeutic strategies. Methods: Eight cases of SPTP, admitted from 1996 to March 2005, were analyzed retrospectively, and all the literature concerning SPTP published in Chinese medical journals from January 1994 to March 2005 were analyzed. Results: A total of 186 cases have been reported since 1995, among which 162 were females (87.1%), with a male to female ratio of 1:6.8. A histogram of the ages at onset illustrated an approximately smooth skewed distribution with a single peak, which was located between 10 and 19 years. The number of diseases located at the pancreatic head is similar to that located at the body and the tail. Malignancy was pathologically diagnosed in 22 cases (11.6%). Major clinical presentations included abdominal pain of varying degree (43.5%) and palpable abdominal masses (37.1%). Treatment strategies include different types of resection, ranging from tumor enucleation to multiple organ resection, and the prognosis is generally good. Conclusions: SPTP occurs predominantly in adolescent and young females, and a satisfactory effect could be achieved with active and appropriate surgeries.

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