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Acinar cell carcinoma, pancreas
Definition: It is a rare tumor that represents less than 2% of all exocrine tumors of the pancreas.
Diagnosis: Although it occurs more often in adults between the 5th and 7th decades of life, a few cases have been reported in children. Males greatly outnumber females. Presenting symptoms are nonspecific, and jaundice is infrequent. Histologically, it can resemble islet cell tumors, pancreatoblastomas, and may also mimic normal pancreatic tissue, but it differs in its ultrastructural and immunohistochemical features. The presentation may feature extensive subcutaneous and intraosseous fat necroses from excessive lipase production by the tumor. It may associate with elevated serum alpha-fetoprotein levels.
Prognosis and Treatment: The survival curves suggest a more aggressive cancer than pancreatic endocrine neoplasms but one that is less aggressive than ductal adenocarcinoma of the pancreas. Those patients who present with localized disease have a much better prognosis than those who present with metastases. There is a high recurrence rate after complete surgical resection, suggesting that micrometastases are present even in localized disease and that adjuvant therapies may be indicated. Chemotherapy and radiation afford disappointing results, however, and novel therapies are needed.
Essential References:
Holen KD et al., Clinical Characteristics and Outcomes From an Institutional Series of Acinar Cell Carcinoma of the Pancreas and Related Tumors. J Clin Oncol 20(24):4673-4678,2002
Ordonez NG: Pancreatic acinar cell carcinoma. Adv Anat Pathol 2001 May;8(3):144-159
Kuerer H et al.: Functioning pancreatic acinar cell carcinoma: immunohistochemical and ultrastructural analyses. Am J Clin Oncol 1997 Feb;20(1):101-107
Hoorens A et al.: Pancreatoblastoma in an adult: its separation from acinar cell carcinoma. Virchows Arch 1994;424(5):485-490
Hsueh C, Kuo TT: Acinar cell carcinoma of the pancreas. Report of two cases with complex histomorphologic features causing diagnostic problems. Int J Pancreatol 1992 Dec;12(3):305-313
Klimstra DS et al.: Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases. Am J Surg Pathol 1992 Sep;16(9):815-837
Itoh T et al.: Acinar cell carcinoma of the pancreas with elevated serum alpha-fetoprotein levels: a case report and a review of 28 cases reported in Japan. Gastroenterol Jpn 1992 Dec;27(6):785-791
Chiou YY et al. Acinar cell carcinoma of the pancreas: clinical and computed tomography manifestations. J Comput Assist Tomogr. 2004 Mar-Apr;28(2):180-6.
Tatli S et al. CT and MRI Features of Pure Acinar Cell Carcinoma of the Pancreas in Adults. AJR Am J Roentgenol. 2005 Feb;184(2):511-9.
Stelow EB et al. Cytology of pancreatic acinar cell carcinoma. Diagn Cytopathol. 2006 May;34(5):367-72.
Riechelmann RP et al. Acinar cell carcinoma of the pancreas. Int J Gastrointest Cancer. 2003;34(2-3):67-72.
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CREATION DATE
Lucio Scopsi: 18/12/2002 8.49.37
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