Solid-pseudopapillary tumor of the pancreas: a surgical enigma?

BACKGROUND: Solid-pseudopapillary tumors (SPTs) of the pancreas have been reported as rare lesions with "low malignant potential" occurring mainly in young women. This study was designed to define the clinicopathological characteristics and the effect of surgical intervention.
METHODS: A retrospective review from January 1985 to July 2000 was performed. Clinicopathological, operative, and survival data were obtained. The Kaplan-Meier method and chi2 analysis were performed. All cases were re-reviewed by a senior pathologist.
RESULTS: During this time, 24 patients were diagnosed as having SPTs (0.9%). Twenty females and four males were identified, with a median age of 39 years (range, 12-79). The median size of the lesions was 8.0 cm (range, 1-20). Two patients' tumors were found to be unresectable at initial presentation because of vascular invasion; both patients have remained alive with disease, one for 13 years and the other 1 year. At a median follow-up of 8 years, one recurrence occurred in 17 patients who underwent complete resection. Microscopic margin positive (P = .26), invasion of surrounding structures (P = .51), and size >5 cm (P = .20) were not significant predictors of survival. Four patients presented with synchronous liver metastasis and underwent resection of the primary tumor and the liver metastasis, with one patient dying of progression of metastatic disease at 8 months, another alive with recurrence in the liver at 6 years, and the last two alive without evidence of disease at 1 month and 11 years.
CONCLUSIONS: SPT occurs predominantly in women (82%), although it can occur in men; all age groups are affected. Complete resection is associated with long-term survival even in the presence of metastatic disease.