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Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females.It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years.The occurrence of infiltrating varieties of 5PT is around 10%-15%.Between 1986 and 2006,282 cystic tumors of the pancreas were observed.Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety.This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst.The tumor invaded a long segment of the portalmesenteric vein confluence,and was removed with a total pancreatectomy,resection of the portal vein and reconstruction with the internal jugular vein.Histological examination confirmed the R-0 resection of the primary SPT,although a vascular invasion was demonstrated.The postoperative course was uneventful,but 32 mo after surgery the patient experienced diffuse liver metastases.Chemotherapy with different drugs was started.The patient is alive and symptom-free,with stable disease,75 mo after surgery.Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature,16 recent patients with tumor relapse after potentially curative resection were also retrieved.The best treatment remains a radical resection whenever possible,even in locally advanced or metastatic disease.The role of chemotherapy,and/or radiotherapy,is still to be defined.