Tumors of the exocrine pancreas in children and adolescents. A clinical and pathologic study of eight cases.

Eight patients with a tumor of the exocrine pancreas were treated or seen in consultation at the Children's Hospital Medical Center over the last 30 years. There were five girls and three boys ranging in age from 15 months to 18 years (average age: 8 years). Tumors were surgically resectable in six of eight cases--three by modified Whipple procedure and three by local resection. The average diameter of tumor was 13.6 cm (range: 3.8-20 cm). There were three adenocarcinomas of duct cell type, two acinar cell carcinomas, two acinar cell adenomas, and a papillary-cystic tumor of low-grade malignancy. One of the adenocarcinomas arose in a field of orthovoltage radiation and another occurred in a patient with tuberous sclerosis. Five of eight patients died, four with regional or distant metastases documented at autopsy. The patient with the papillary-cystic tumor experienced several local recurrences but was alive 14 years after initial surgery. Data from the literature indicate that acinar cell tumors are proportionately more common in children than in adults and have a somewhat better prognosis than tumors of duct cell type. Further reporting of cases with long-term follow-up is encouraged in order to gain clearer perspective of nonendocrine tumors of the pancreas in childhood.