Acinar cell carcinoma of pancreas (Q94573)

Acinar cell carcinoma is a malignant epithelial neoplasm composed of cells with morphologically resemblance to acinar cells and with evidence of pancreatic exocrine enzyme production. Most acinar cell carcinomas present with nonspecific symptoms. They generally compress rather than infiltrate into adjacent structures. The lipase hypersecretion syndrome with clinical symptoms including subcutaneous fat necrosis and polyarthralgia occurs in 10-15% of patients. Grossly, most acinar cell carcinomas are large, well-demarcated, and soft and fleshy. Microscopically, they are cellular with little desmoplastic stroma. Immunohistochemical labeling will reveal the expression of exocrine enzymes. They are very aggressive and metastases are found in half of patients at presentation.