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

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.