Solid pseudopapillary tumor of the pancreas (Frantz's tumor): Two case reports and a review of the literature

Abstract

Introduction: Solid pseudopapillary tumor of the pancreas is extremely rare in children; it usually occurs in young women between 18 and 35 years of age. It comprises less than 3 % of pancreatic tumors. It is of low malignancy; however, it may be locally aggressive. Surgical resection is the treatment of choice and its prognosis is excellent. Case presentation: Two Caucasian girls, 15 and 12 years of age were diagnosed with tumor of the pancreas. The first patient had severe abdominal pain. In the second case the tumor was asymptomatic, detected incidentally during ultrasound. Computed tomography confirmed pancreatic mass. In the first case, apart from the tumor located in the head and the body of her pancreas, focal change in her right kidney was found, which was an indication to biopsy that confirmed solid pseudopapillary tumor. In the second patient the tumor was located in the body of her pancreas, with portal vein occlusion and well-developed collateral circulation. In the first patient a pancreatoduodenectomy (Traverso-Longmire) was performed; there was no mass in her right kidney. In the second case, distal pancreatectomy and splenectomy were performed. In both cases histopathology revealed solid pseudopapillary tumor resected radically. Our first patient's postoperative course was uneventful. In the second case, her postoperative course was complicated by necrosis of the remaining pancreatic head that needed pancreatoduodenectomy. Follow-up at 28 and 26 months revealed no evidence of tumor recurrence or metastases on magnetic resonance imaging. Conclusions: Typical radiological appearance of solid pseudopapillary tumor is an indication for surgery. The treatment of choice is tumor resection with sparing of pancreatic tissue. In one of our two cases we performed a preoperative biopsy because of an uncharacteristic mass in her right kidney. In our second patient, necrosis of her spared pancreatic head meant that we could not preserve pancreatic tissue. Our whole diagnostic process, treatment and possible complications analysis should be of interest and noteworthy not only to surgeons as the treatment of choice is radical resection, but also to pediatric oncologists because of differentiation from other pancreatic tumors in children.