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WHO Classification of Tumours
Acinar cell carcinoma
Pancreas


Definition

Acinar cell carcinoma is a malignant epithelial neoplasm composed of cells with morphological resemblance to acinar cells and with evidence of pancreatic exocrine enzyme production. Cases with significant endocrine or ductal components (i.e. > 25% of the neoplasm) are regarded as mixed carcinomas (mixed acinar-neuroendocrine carcinoma, mixed acinar-ductal carcinoma, or mixed acinarneuroendocrine- ductal carcinoma)
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Stelow EB, Bardales RH, Shami VM, Woon C, Presley A, Mallery S, Lai R, Stanley MW (2006)
Cytology of pancreatic acinar cell carcinoma.
Diagn Cytopathol 34: 367-72




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Stelow EB, Shaco-Levy R, Bao F, Garcia J, Klimstra DS (2010)
Pancreatic acinar cell carcinomas with prominent ductal differentiation: Mixed acinar ductal carcinoma and mixed acinar endocrine ductal carcinoma.
Am J Surg Pathol 34: 510-8




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Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



. Rare cases with macroscopic, nondegenerative cyst formation have been reported as acinar cell cystadenocarcinoma
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Cantrell BB, Cubilla AL, Erlandson RA, Fortner J, Fitzgerald PJ (1981)
Acinar cell cystadenocarcinoma of human pancreas.
Cancer 47: 410-6




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Colombo P, Arizzi C, Roncalli M (2004)
Acinar cell cystadenocarcinoma of the pancreas: report of rare case and review of the literature.
Hum Pathol 35: 1568-71




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Hoorens A, Lemoine NR, McLellan E, Morohoshi T, Kamisawa T, Heitz PU, Stamm B, Rüschoff J, Wiedenmann B, Klöppel G (1993)
Pancreatic acinar cell carcinoma. An analysis of cell lineage markers, p53 expression, and Ki-ras mutation.
Am J Pathol 143: 685-98




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JACKSON SH, VARLEY H (1952)
Carcinoma of the pancreas associated with fat-necrosis.
Lancet 2: 962-7




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Stamm B, Burger H, Hollinger A (1987)
Acinar cell cystadenocarcinoma of the pancreas.
Cancer 60: 2542-7



.

They represent 1–2% of all exocrine pancreatic neoplasms in adults

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Hoorens A, Lemoine NR, McLellan E, Morohoshi T, Kamisawa T, Heitz PU, Stamm B, Rüschoff J, Wiedenmann B, Klöppel G (1993)
Pancreatic acinar cell carcinoma. An analysis of cell lineage markers, p53 expression, and Ki-ras mutation.
Am J Pathol 143: 685-98




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Klimstra DS, Heffess CS, Oertel JE, Rosai J (1992)
Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.
Am J Surg Pathol 16: 815-37



and 15% of those in children
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Hoorens A, Lemoine NR, McLellan E, Morohoshi T, Kamisawa T, Heitz PU, Stamm B, Rüschoff J, Wiedenmann B, Klöppel G (1993)
Pancreatic acinar cell carcinoma. An analysis of cell lineage markers, p53 expression, and Ki-ras mutation.
Am J Pathol 143: 685-98




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Holen KD, Klimstra DS, Hummer A, Gonen M, Conlon K, Brennan M, Saltz LB (2002)
Clinical characteristics and outcomes from an institutional series of acinar cell carcinoma of the pancreas and related tumors.
J Clin Oncol 20: 4673-8




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Klimstra DS, Heffess CS, Oertel JE, Rosai J (1992)
Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.
Am J Surg Pathol 16: 815-37



, but most cases occur in late adulthood. Immunohistochemical detection of the production of pancreatic exocrine enzymes (e.g. trypsin, chymotrypsin, lipase, and elastase) is helpful in confirming the diagnosis. Acinar cell carcinomas are aggressive; however, outcome is better than that for stage-matched infiltrating ductal adenocarcinomas, with 5-year survival figures of 25–50% depending on stage at diagnosis
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Kitagami H, Kondo S, Hirano S, Kawakami H, Egawa S, Tanaka M (2007)
Acinar cell carcinoma of the pancreas: clinical analysis of 115 patients from Pancreatic Cancer Registry of Japan Pancreas Society.
Pancreas 35: 42-6




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Matos JM, Schmidt CM, Turrini O, Agaram NP, Niedergethmann M, Saeger HD, Merchant N, Johnson CS, Lillemoe KD, Grützmann R (2009)
Pancreatic acinar cell carcinoma: a multi-institutional study.
J Gastrointest Surg 13: 1495-502




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Schmidt CM, Matos JM, Bentrem DJ, Talamonti MS, Lillemoe KD, Bilimoria KY (2008)
Acinar cell carcinoma of the pancreas in the United States: prognostic factors and comparison to ductal adenocarcinoma.
J Gastrointest Surg 12: 2078-86




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Wisnoski NC, Townsend CM, Nealon WH, Freeman JL, Riall TS (2008)
672 patients with acinar cell carcinoma of the pancreas: a population-based comparison to pancreatic adenocarcinoma.
Surgery 144: 141-8



. There are reports of survival for several years in the presence of metastatic disease, and responses to chemotherapy have been noted
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Klimstra DS, Heffess CS, Oertel JE, Rosai J (1992)
Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.
Am J Surg Pathol 16: 815-37




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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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