Evaluation of childhood solid pseudopapillary tumors of the pancreas

OBJECTIVE: Solid pseudopapillary tumor (SPT) of the pancreas is an extremely rare primary tumor in the pediatric age group. It has a low malignant potential and the prognosis is good if radical resection of the tumor is performed. Local recurrence and distant metastasis has only rarely been reported following incomplete resection. METHODS: A retrospective review of the medical records of 6 patients diagnosed as SPT according to a histopathological examination at the Children's Hospital of Erciyes University School of Medicine between 2010 and 2017 was performed. Demographic characteristics, tumor localization and size, diagnostic method, immunohistochemical staining features, and medical and surgical treatments employed were recorded. RESULTS: There were 4 girls and 2 boys with the diagnosis of SPT included in this study. The mean age of the patients was 14 years (min-max: 13-16 years). The most common presenting complaint was abdominal pain. The mass lesion was at the head of the pancreas in 3 cases (50%) and the tail of the pancreas in the remaining 3 patients (50%). A Whipple procedure was performed in 3 cases, a distal pancreatectomy in 1, a distal pancreatectomy plus splenectomy in 1, and a subtotal pancreatectomy in 1patient. Immunohistochemistry revealed positive staining for beta-catenin, keratin, CD56, vimentin, and CD10 in all cases. CONCLUSION: SPT is a rarely seen pancreatic mass with low rate of malignancy. Diagnosis may be delayed due to its asymptomatic nature in most cases and a lack of descriptive symptoms. The survival rate is quite high after radical resection.