Department of Medicine, Icahn School of Medicine at Mount Sinai, Mount Sinai St. Luke's and Mount Sinai West, New York, NY, 10019, USA.
Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USA.
Drugs. 2020 May;80(7):647-669. doi: 10.1007/s40265-020-01304-0.
Pancreatic ductal adenocarcinoma (PDAC) is typically diagnosed at an advanced stage, with systemic therapy being the mainstay of treatment. Survival continues to be limited, typically less than 1 year. The PDAC microenvironment is characterized by a paucity of malignant epithelial cells, abundant stroma with predominantly immunosuppressive T cells and myelosuppressive-type macrophages (M2), and hypovascularity. The current treatment options for metastatic PDAC are modified (m)FOLFIRINOX /FOLFIRINOX or nab-paclitaxel and gemcitabine in patients with good performance status (PS) (ECOG 0-1/KPS 70-100%) and gemcitabine with or without a second agent for those with ECOG PS 2-3. New therapies are emerging, and the current guidelines endorse both germline and somatic testing in PDAC to evaluate actionable findings. Important themes related to new therapeutic approaches include DNA damage repair strategies, immunotherapy, targeting the stroma, and cancer-cell metabolism. Targeted therapy alone (outside small genomically defined subsets) or in combination with standard cytotoxic therapy, thus far, has proven disappointing in PDAC; however, novel therapies are evolving with increased integration of genomic profiling along with a better understanding of the tumor microenvironment and immunology. A small but important sub-group of patients have some of these agents available in the clinics for use. Olaparib was recently approved by the US Food and Drug Administration for maintenance therapy in germline BRCA1/2 mutated PDAC following demonstration of survival benefit in a phase 3 trial. Pembrolizumab is approved for patients with defects in mismatch repair/microsatellite instability. PDAC with wild-type KRAS represents a unique subgroup who have enrichment of potentially targetable oncogenic drivers. Small-molecule inhibitors including ERBB inhibitors (e.g., afatinib, MCLA-128), TRK inhibitors (e.g., larotrectinib, entrectinib), ALK/ROS inhibitor (e.g., crizotinib), and BRAF/MEK inhibitors are in development. In a small subset of patients with the KRASG12C mutation, a KRASG12C inhibitor, AMG510, and other agents are being investigated. Major efforts are underway to effectively target the tumor microenvironment and to integrate immunotherapy into the treatment of PDAC, and although thus far the impact has been modest to ineffective, nonetheless, there is optimism that some of the challenges will be overcome.
胰腺导管腺癌(PDAC)通常在晚期诊断,系统治疗是主要的治疗方法。生存时间仍然有限,通常不到 1 年。PDAC 的微环境特征是恶性上皮细胞稀少,富含基质,主要是具有免疫抑制作用的 T 细胞和骨髓抑制型巨噬细胞(M2),以及血管生成不足。转移性 PDAC 的当前治疗选择是改良(m)FOLFIRINOX/FOLFIRINOX 或 nab-紫杉醇加吉西他滨用于身体状况良好的患者(ECOG 0-1/KPS 70-100%),对于 ECOG PS 2-3 的患者,吉西他滨加或不加第二种药物。新的治疗方法正在出现,目前的指南在 PDAC 中既支持种系测试也支持体细胞测试,以评估可操作的发现。与新的治疗方法相关的重要主题包括 DNA 损伤修复策略、免疫疗法、靶向基质和癌细胞代谢。单独的靶向治疗(不在小的基因组定义亚组之外)或与标准细胞毒性治疗联合使用,迄今为止,在 PDAC 中的效果令人失望;然而,随着基因组分析的整合以及对肿瘤微环境和免疫学的更好理解,新的治疗方法正在不断发展。一小部分患者在临床上有一些这些药物可供使用。奥拉帕利最近被美国食品和药物管理局批准用于种系 BRCA1/2 突变的 PDAC 的维持治疗,因为在一项 3 期试验中显示了生存获益。派姆单抗被批准用于错配修复/微卫星不稳定缺陷的患者。野生型 KRAS 的 PDAC 代表了一个独特的亚组,其中富含潜在可靶向的致癌驱动基因。包括 ERBB 抑制剂(如阿法替尼、MCLA-128)、TRK 抑制剂(如拉罗替尼、恩曲替尼)、ALK/ROS 抑制剂(如克唑替尼)和 BRAF/MEK 抑制剂在内的小分子抑制剂正在开发中。在一小部分 KRASG12C 突变患者中,KRASG12C 抑制剂 AMG510 和其他药物正在被研究。目前正在进行重大努力,以有效靶向肿瘤微环境,并将免疫疗法整合到 PDAC 的治疗中,尽管到目前为止,其影响是适度的,甚至是无效的,但人们仍然乐观地认为,一些挑战将得到克服。