Abstract

Acinar cell carcinoma pancreas is an epithelial, malignant neoplasm of exocrine pancreas comprised of cells resembling pancreatic acinar cells which are immune reactive to BCL10 and trypsin. Tumefaction arises due to accumulated genetic alterations, chromosomal instability or allelic copy number variation. Cogent clinical symptoms as abdominal pain, dorso-lumbar pain, loss of weight, nausea or vomiting or subcutaneous fat necrosis may ensue. Grossly, a solid, enlarged, well circumscribed, partially encapsulated tumefaction of fleshy consistency is encountered. Neoplastic cells demonstrate granular, eosinophilic cytoplasm pervaded with zymogen granules stainable with periodic acid Schiff’s (PAS) stain with diastase resistance, uniform nuclei and a singular, prominent nucleolus. Scanty and fibrous encompassing stroma exhibits foci of perineural and vascular invasion. Acinar cell carcinoma pancreas is immune reactive to keratins as CK7, CK8, CK18, CK19, BCL10, trypsin nuclear beta catenin or CD200. Computerized tomography (CT) and magnetic resonance imaging (MRI) delineates an enlarged tumefaction with well-defined perimeter, an exophytic pattern of tumour evolution and heterogeneous image enhancement. Surgical resection, chemotherapy with gemcitabine or radiofrequency ablation are appropriate modes of therapy.

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