Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases

Abstract

BACKGROUND/PURPOSE: Solid pseudopapillary tumor (SPT) is a very rare form of childhood pancreatic tumor. This study was intended to analyze the clinicopathologic characteristics of this tumor in childhood. METHODS: We retrospectively reviewed the medical records of patients who were pathologically diagnosed with SPT between March 1991 and March 2005. RESULTS: Twenty-three patients underwent an operation and were pathologically diagnosed with SPT. Five (22%) were male, with a male-to-female ratio of 1:3.6. The initial signs and symptoms were upper abdominal pain in 20 patients (87%), palpable abdominal mass in 8 (35%), and dyspepsia in 6 (26%). Four patients (17%) had a history of abdominal trauma. Tumors were located in the pancreatic head (30%), body (13%), tail (44%), and both body and tail (13%). Operative procedures performed were pylorus-preserving pancreaticoduodenectomy (n = 6, 26.1%), distal pancreatectomy (n = 7, 30.4%), distal pancreatectomy with splenectomy (n = 7, 30.4%). The median follow-up period was 62 months (range, 6-175 months). One patient showed multiple liver metastasis 3 months after the initial operation and required adjuvant chemotherapy. All other patients were alive at their most recent follow-up with no evidence of recurrence or distant metastasis. CONCLUSIONS: Complete resection of an SPT is usually curative and patients can survive a long period after the operation.