伴有腺泡分化的胰腺癌很少见,占成人胰腺肿瘤的1%-2%;它们包括胰腺腺泡细胞癌(PACC),胰母细胞瘤,和混合分化癌。PACC患者的预后比胰腺导管腺癌好,但比胰腺神经内分泌肿瘤差。总生存期的报告范围为18至47个月。关于PACCs的文献综述包括对一系列70多名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。辅助治疗的疗效尚不清楚。转移性PACCs通常是不可治愈的,并且用全身化疗治疗。他们对不同方案的化疗有中等反应,在病例报告/系列中显示出不同程度的反应。这些方案中的大多数被开发用于治疗患有胰腺导管腺癌或结直肠腺癌的患者。PACC分子谱分析的综述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具有多个体细胞突变,其中一些可用药物靶向。因此,PACC的分子谱分析应该是难治性PACC患者的一种选择。
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.