Diagnosis and treatment of papillary cystic tumor of the pancreas in children.

We report on 3 children with a papillary cystic tumor of the pancreas. The patients presented with abdominal pain or a palpable tumor. All of them underwent complete tumor resection and spleen preservation was possible in 2 patients. Histologic examination showed a papillary cystic tumor of the pancreas. This tumor is characterized by a non-invasive growth pattern and metastasis is very rare. The boy described is the youngest male patient reported in the English literature. All patients are alive 6-51 months after diagnosis. Recognition of this entity is important because treatment differs from that of other pancreatic malignancies. Tumor resection without large safety margins is adequate for treatment. Therefore, preservation of pancreatic tissue and the spleen should be attempted in every child.