Citation, DOI, disclosures and article data
Citation:
Iqbal S, Campos A, Sharma R, et al. Pancreatic acinar cell carcinoma. Reference article, Radiopaedia.org (Accessed on 24 Apr 2024) https://doi.org/10.53347/rID-87933
Pancreatic acinar cell carcinomas are rare exocrine neoplasms that comprise ~1% of all pancreatic tumors. This tumor shows more aggressive behavior than the far more common adenocarcinoma 1,3,4.
This tumor is most common in pediatric (8-15 years) and adult (60 years) populations. There is a male predominance 6.
Presenting symptoms are non-specific including weight loss, abdominal and/or back pain, and nausea and vomiting 6. Pancreatic acinar cell carcinoma can present with lipase hypersecretion syndrome (serum lipase levels >10,000 U/dL), which can result in presentation of Schmid's triad with subcutaneous fat necrosis, polyarthropathy, and serum eosinophilia 2,3,5,6.
CT
on contrast-enhanced CT, generally presents as an enhancing, ovoid, solid pancreatic tumor
variable hypo/hyperattenuating mass relative to the pancreas, with well-distinguished margins
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non-biliary duct dilation
hypovascular tumor with adjacent organ invasion
internal calcification (50% of cases)
tumor encapsulation
peripancreatic lymph nodes
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Treatment and prognosis
In cases of disease confinement to the pancreas, surgical resection is curative and in cases of distant metastasis, surgery followed by chemotherapy and radiotherapy is the treatment of choice 1,2,4.
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1. Barat M, Dohan A, Gaujoux S et al. Computed Tomography Features of Acinar Cell Carcinoma of the Pancreas. Diagn Interv Imaging. 2020;101(9):565-75. doi:10.1016/j.diii.2020.02.007 - Pubmed
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2. Mustafa S, Hruban R, Ali S. Acinar Cell Carcinoma of the Pancreas: A Clinicopathologic and Cytomorphologic Review. J Am Soc Cytopathol. 2020;9(6):586-95. doi:10.1016/j.jasc.2020.04.005 - Pubmed
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3. Miksch R, Schiergens T, Weniger M et al. Pancreatic Panniculitis and Elevated Serum Lipase in Metastasized Acinar Cell Carcinoma of the Pancreas: A Case Report and Review of Literature. World J Clin Cases. 2020;8(21):5304-12. doi:10.12998/wjcc.v8.i21.5304 - Pubmed
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4. Duorui N, Shi B, Zhang T, Chen C, Fang C, Yue Z, Wu P, Wu Z, Huang X, Li M. The contemporary trend in worsening prognosis of pancreatic acinar cell carcinoma: A population-based study. (2020) PloS one. 15 (12): e0243164. doi:10.1371/journal.pone.0243164 - Pubmed
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5. Zong Y, Qi C, Peng Z, Shen L, Zhou J. Patients With Acinar Cell Carcinoma of the Pancreas After 2005: A Large Population Study. Pancreas. 2020;49(6):781-7. doi:10.1097/MPA.0000000000001573 - Pubmed
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6. Calimano-Ramirez L, Daoud T, Gopireddy D et al. Pancreatic Acinar Cell Carcinoma: A Comprehensive Review. World J Gastroenterol. 2022;28(40):5827-44. doi:10.3748/wjg.v28.i40.5827 - Pubmed
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