Solid pseudopapillary neoplasm of the pancreas in children: a clinical and pathological study of 16 cases  期刊论文  

Introduction: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic neoplasm with malignant potential. There are many unique differences between children and adults in solid pseudopapillary neoplasm of the pancreas. Our study aimed to illustrate the clinical and pathological features of solid pseudopapillary neoplasm of the pancreas in children. Method: The clinical and pathological data of 16 pediatric cases of solid pseudopapillary neoplasm of the pancreas were retrieved and reviewed from the medical records in the Kunming Children's Hospital. Results: There were 11 girls and 5 boys, the sex ratio was 2.2:1 (female-to-male); the median age was 9.5 years, and the average age was 9.56 +/- 3.65 years (ranging from 3 to 15 years). The chief complaint was abdominal pain (8/16) and a mass in the abdomen (3/16). The median size of the tumors was 7.9 cm in maximum diameter (ranging from 2.8 to 16.4 cm). The lesions were located at the head (7 cases, 43.75%), the body (4 cases, 25%), the tail (1 case, 6.25%), both the head and the body (1 cases, 6.25%), and both the body and the tail (3 cases, 18.75%). 9 cases (including 4 boys) of solid pseudopapillary neoplasm of the pancreas were cystic and solid (56.25%), 6 cases were purely solid (37.5%), and only 1 case was purely cystic (6.25%). Distal pancreatectomy was the choice for the body/tail tumors (50%), pancreaticoduodenectomy and total mass excision for the head tumors (43.75%), and total pancreatectomy for the head and body tumors (6.25%). Histologically, pseudopapillaes, solid cell sheets surrounded by fibrovascular stroma, nuclear grooves, areas of myxoid change and haemorrhage were the rudimentary histological patterns of solid pseudopapillary neoplasm of the pancreas. On immunohistochemistry the tumors variably expressed vimentin, neuron-specific enolase, alpha-1-antitrypsin, progesterone receptor, beta-catenin, CD99 (dot-like intracytoplasmatic), cytokeratin, synaptophysin, Ki-67 (2-4%), and were negative to chromogranin A. The prognosis was satisfying. The average follow-up was 22.88 +/- 16.69 months (ranging from 3 to 61 months) and all the patients were event-free after operation. Conclusion: Solid pseudopapillary neoplasm of the pancreas was a borderline tumor with malignant potential and was uncommon in children. Compared with the adults, the pediatric patients showed many different characteristics: gender disparity, main clinical manifestations, the tumor location distribution, the size and configuration, and degenerative changes. Immunohistochemical markers such as beta-catenin and CD99 were useful for the diagnosis. Although with malignant potential, metastases and recurrence were rarely observed in children.