Carcinoma of the pancreas with endocrine component in childhood. A case report.

A case of pancreatic tumor in a six-year-old girl is presented. The tumor had histologic characteristics of acinar cell carcinoma with endocrine component. Grossly, it was encapsulated and attached to the tail of the pancreas, measuring 8 cm in the greatest diameter. Histologically, the tumor was composed of medium-sized tumor cells, with mild pleomorphism showing mainly acinar structures. Many of these tumor cell contained fine granules that were periodic acid-Schiff positive, diastase resistant, and positive with dimethylaminobenzaldehyde nitrite strain for tryptophan, and some contained granules that were positive with Grimelius stain and positive with peroxidase-antiperoxidase technic for gastrin. Electron microscopy revealed two types of membrane-bound granules in the tumor cells. The larger granules measured 400-700 nm in diameter and appeared to be zymogen granules, while the smaller ones measured 100-200 nm in diameter and appeared to be neuroendocrine granules. Some cells contained both granules. The postoperative course of the patient was excellent, and she was alive and well 13 years after operation. This may be the second reported case of acinar-endocrine cell tumor of the pancreas.