Solid pseudopapillary neoplasms-experience from a tertiary care centre

Abstract

Introduction: Solid pseudopapillary neoplasms (SPN/FRANTZ TUMOUR) of the pancreas are rare neoplasms of lo w grade malignant potential which were first described in 1959 by Frantz. These account for 0.13-2.7% of pancreatic neoplasms and approximately 13% of surgically resected cystic lesions of the pancreas. We present our experience with these rare tumors. Methods: Total 406 patients with pancreatic tumours were ad mitted in our department during the 10 year period (Between 2007 and 2017) were reviewed, only 18 were diagnosed as having SPN (4.4%). Clinicopathological details, intervention done and follow up of all the cases were studied and reported here. Results: 17 patients were wo man and1was Man with median age of 23 years (range 11 to 54 years). The tumor size ranged from 3.8 to17cm (average 6.4 cm). 12 patients presented with pain in the abdomen, 4 presented with a painless mass, 1 was detected incidentally and1presented with Malena. In 7 patients the tumor was in the pancreatic head, in 3 it was in the neck, and in the remaining 8 it is in the body and tail. CECT was done in all cases. 8 patients underwent Distal pancreatectomy with splenectomy, 1 underwent a PPPD, 6 patients required classical Whipple operation. 3 underwent central pancreatectomy. Immuno histochemistry showed positivity for beta catenin, vimentin, PR receptor and chromogranin negativity. All 18 patients were free of d isease in a median follow-up period of 32 months (range 6-84) months. Conclusion: SPNs are rare neoplasms, typically affecting young women without notable symptoms, with a low malignant potential but excellent prognosis. Radical surgical resection with clear margins is the treatment of choice.