Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
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DOI：
10.3748/wjg.v23.i45.7945
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发表时间：
2017-12-07
影响因子：
4.3
通讯作者：
Von Hoff D
中科院分区：
文献类型：
--
作者：
Al-Hader A;Al-Rohil RN;Han H;Von Hoff D
胰腺腺泡分化少见，占成人胰腺肿瘤的1%-2%；它们包括胰腺腺泡细胞癌（PACC）、胰腺母细胞瘤和混合分化癌。PACC患者预后好于胰腺导管腺癌，但差于胰腺神经内分泌肿瘤。总生存期从18个月到47个月不等。关于PACCs的文献综述包括对70多例患者的全面基因组分析和全外显子组测序，以及包括免疫组织化学在内的其他诊断研究。手术切除PACC是局部和可切除肿瘤的首选治疗方法。辅助治疗的效果尚不清楚。转移性PACCs通常无法治愈，需要全身化疗。他们对不同方案的化疗反应中等，在病例报告/系列中表现出不同程度的反应。这些方案大多是为了治疗胰腺导管腺癌或结直肠腺癌患者而开发的。对PACC分子谱的回顾显示了许多基因改变，如：SMAD4、BRAF、BRCA2、TP53、RB1、MEN1、JAK-1、BRCA-1、BRCA-2和DNA错配修复异常。PACCs有多个体细胞突变，其中一些是可用药物靶向的。因此，对于难治性PACC患者，分子谱分析应该是一种选择。
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.
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