Acinar Cell Carcinoma of the Pancreas: Clinical and Cytomorphologic Characteristics.
- Author:
Adam D TOLL
1
;
Ralph H HRUBAN
;
Syed Z ALI
Author Information
1. Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA. sali@jhmi.edu
- Publication Type:Review
- Keywords:
Carcinoma, acinar cell;
Pancreas
- MeSH:
Acinar Cells;
Adenocarcinoma;
Carcinoma;
Carcinoma, Acinar Cell;
Cytoplasm;
Humans;
Jaundice;
Lipase;
Liver;
Lymph Nodes;
Male;
Neuroendocrine Cells;
Pancreas;
Pancreatic Ducts;
Prognosis
- From:Korean Journal of Pathology
2013;47(2):93-99
- CountryRepublic of Korea
- Language:English
-
Abstract:
Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.