Solid pseudopapillary tumor of the pancreas: a case series of 11 children

This study aimed to analyze the clinical data, pathologic features and surgical management about solid pseudopapillary tumor of the pancreas (SPTP) in children. The clinical data and pathological findings from 11 children with SPTP were retrospectively analyzed, who were diagnosed and treated in three tertiary academic centers between January 2001 and December 2015. The 11 children consisted of 10 females and 1 male, of median age at operation of 11.4 years old (range from 3.7 to 16.3 years old). The clinical symptoms were non-specific, and mainly manifested upper abdominal pain or discomfort. The CT results of all patients were similar, and the feature of SPTP was solid or mixed solid and cystic. The neoplasm was localized in the pancreatic head/neck in 4 patients, and in the body in 3 patients, and in the tail in 4 patients, which were all confirmed during abdominal operations. The diameter of these lesions ranged from 2.8 to 17.3 cm, without abdominal cavity metastasis or hepatic metastases. All of the tumors were resected successfully, which included 2 pancreaticoduodenectomyies (Whipple-Child), 4 local resections, 1 segmental pancreatectomy, 2 distal pancreatosplenectomy, 2 spleen-preserving distal pancreatectomies. The follow-up of all cases ranged from 8 months to 14 years, and no tumor recurrence or metastasis was observed in these SPTP children. The results indicated that all tumors were positive for the immunohistochemical staining of beta-catenin, progesterone receptor (PR), vimentin, and CD99. However, all the tumors were negative for the immunohistochemical staining of E-cadherin and cytokeratin 7 expression. CT examination combined with age and sex profile should be sufficient for children SPTP diagnosis. Surgery was the main treatment choice Children with SPTP have an excellent prognosis after surgical excision. Surgery strategy should be determined according to preoperative CT examination, intraoperative findings of tumor location, capsule integrity, and invaded surrounding tissues. Immunohistochemical staining of E-cadherin/beta-catenin, PR, vimentin, and CD99 may help the diagnosis, though their expressions were found to have the indistinct complex immunophenotypes.