Acinar cell carcinoma of the pancreas with and without endocrine differentiation

Abstract

Background: Acinar cell carcinoma (ACC) is a rare pancreatic neoplasm, representing 1% of exocrine tumours and containing a variable endocrine component. Three recent cases of ACC are reported. Case outlines: A 72-year-old man with painless obstructive jaundice had a 5-cm mass in the head of pancreas resected by Whipple's operation; histopathological examination showed a typical ACC. A 33-year-old man with weight loss and abnormal liver function had a dilated biliary tree but no mass on imaging. Pylorus-preserving pancreatoduodenectomy was performed, and histology showed a mixed acinar-neuroendocrine tumour. A 56-year-old man with weight loss and a palpable mass had a 15-cm mass in the distal body of pancreas, which was resected en bloc with the spleen and adherent stomach; it was a cystic ACC. Results: Two patients are alive and free of disease at 30 months and 15 months, while the third patient with locally advanced disease died of myocardial infarction at 9 weeks. Discussion: Acinar structures are the hallmark of this neoplasm, which carries a better survival rate than ductal cancer. Surgical excision prolongs survival and offers the best chance of cure.