A Pediatric Case of Acinar Cell Carcinoma of the Pancreas

Abstract

Acinar cell carcinoma of the pancreas is an exocrine malignant neoplasm with features of acinar cell differentiation. It accounts for about 1% to 2% of all pancreatic neoplasms, and pediatric cases are extremely rare.
A 12-year-old girl presented with complaints of vomiting, weight loss, and general malaise was examined. Abdominal CT showed a mass measuring 47 mm from the pancreas head with multiple hepatic and lung metastases. Tumor biopsy revealed a pancreatic acinar cell carcinoma. She was treated with a combination chemotherapy consisting of intravenous infusion of gemcitabine (GEM) and oral administration of tegafur-gimestat-otastat potassium (TS-1). Although progression of the disease was slowed, she suffered from the side effects of GEM, such as fever and general discomfort. Therefore, GEM was replaced with TS-1 monotherapy. The patient’s lung metastases progressed, and she died 496 days after treatment. We reviewed 42 cases of pancreatic acinar cell carcinomas. Some patients who underwent surgical resection achieved long-term survival; however, chemotherapies employed were ineffective against acinar cell carcinomas, and the prognosis of patients with metastasis was very poor.