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Introduction: Acinar cell carcinoma is a rare high-grade aggressive neoplasm of pancreas showing acinar cell differentiation. It makes up 1%-2% of all pancreatic neoplasms in adults and 15% in children.

Gross Pathology:: Acinar cell carcinomas are more common in the head of the pancreas, but can arise anywhere in the organ. They are solitary, well-circumscribed, at least partially encapsulated, and large solid tumors (8-10 cm average). The cut surface is fleshy, friable, homogenous pink and may have areas of hemorrhage and necrosis. Some cases have a prominent cystic component (acinar cell cystadenocarcinomas).

This image shows a large acinar cell carcinoma in the tail of the pancreas. There is extensive cystic degeneration. Spleen is seen at the back. Image courtesy of: Dr. Laura G. Pastrian, Spain; used with permission.
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