Surgery

Treatment of solid pseudopapillary tumor of the pancreas is primarily surgical. However, preoperative and operative spillage is not unusual.[9] Whipple procedures (pancreaticoduodenectomy) are often necessary, but non-Whipple, pancreatic-sparing resections may be possible with a pancreatico-jejunostomy procedure. Surgery is usually curative, although local recurrences occur in 5% to 15% of cases.[4]

A retrospective review of the Italian Pediatric Rare Tumor Registry identified 43 pediatric patients diagnosed with solid pseudopapillary tumor of the pancreas between 2000 and 2018.[10][Level of evidence C1] The median age at diagnosis was 13.2 years (range, 7–18 years). Only one patient presented with metastatic disease.

A study identified 38 children aged 0 to 18 years who were diagnosed with pseudopapillary solid tumor of the pancreas between 2008 and 2022 in a German registry.[11] Patients were a median age of 14.5 years at diagnosis (range, 8–18 years), with a female preponderance (81.6%). The pancreatic tail was the most frequent location of the tumor. All patients underwent surgical resection.