Abstract:
:Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC's molecular profiling showed a number of gene alterations such as: SMAD4, BRAF, BRCA2, TP53, RB1, MEN1, JAK-1, BRCA-1, BRCA-2, and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.
journal_name
World J Gastroenteroljournal_title
World journal of gastroenterologyauthors
Al-Hader A,Al-Rohil RN,Han H,Von Hoff Ddoi
10.3748/wjg.v23.i45.7945subject
Has Abstractpub_date
2017-12-07 00:00:00pages
7945-7951issue
45eissn
1007-9327issn
2219-2840journal_volume
23pub_type
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doi:10.3748/wjg.v23.i6.964
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pub_type: 杂志文章,随机对照试验
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pub_type: 杂志文章
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更新日期:2014-04-28 00:00:00
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pub_type: 杂志文章
doi:10.3748/wjg.v16.i2.275
更新日期:2010-01-14 00:00:00
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journal_title:World journal of gastroenterology
pub_type: 临床试验,杂志文章
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更新日期:2004-12-15 00:00:00
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pub_type: 杂志文章
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更新日期:2009-07-07 00:00:00
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journal_title:World journal of gastroenterology
pub_type: 杂志文章
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更新日期:2009-03-28 00:00:00
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pub_type: 杂志文章
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更新日期:2009-02-07 00:00:00
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pub_type: 杂志文章,评审
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pub_type: 社论,评审
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pub_type: 杂志文章
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journal_title:World journal of gastroenterology
pub_type: 杂志文章
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pub_type: 杂志文章
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