Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature

Abstract

Solid pseudopapillary tumor (SPT) of the pancreas is an infrequent neoplasm of low malignant potential, first described by Frantz in 1933 and representing less than 3% of all exocrine tumors. SPT is rare in children, accounting for 6% of all cases and shows different clinical features compared with adults. Here, a pediatric case is reported and a review of the Literature is provided. A 15-year-old girl with a 12 × 14 × 10 cm solid mass growing from the tail and the body of the pancreas, involving spleen, left adrenal gland and kidney, stomach and some bowel loops, was referred for surgical treatment. A distal pancreasectomy with splenectomy was performed. Histopathological examination revealed that the tumor was a 14-cm well-circumscribed solid mass, with pseudopapillary cell architecture, showing strong cellular immunoreactivity for alpha-1 antitrypsin, vimentin, neurone-specific enolase, progesterone receptors and in part to CD10 and CAM 5.2, but not to sinaptofisin and chromogranin. A 24-month post-surgical follow-up after successful surgical resection showed no evidence of recurrent disease. SPT shows different clinical features in childhood. High survival rates can be achieved in most cases, warranting aggressive treatments even in metastatic disease.