Acinar cell carcinoma of the pancreas

Acinar cell carcinoma (ACC) is a rare tumor that constitutes 1% of pancreatic neoplasms. ACC is defined as a carcinoma exhibiting pancreatic enzyme production by neoplastic cells. Clinical presentation is usually related to either local spread or metastasis. In this Radiology–Pathology Conference, the clinical presentation and imaging findings of a patient with ACC of the pancreas, along with the differential diagnosis, are reviewed.