Laboratory of Molecular Pathology, Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian 1, 20133 Milan, Italy.
Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian 1, 20133 Milan, Italy.
Int J Mol Sci. 2019 Nov 19;20(22):5817. doi: 10.3390/ijms20225817.
Background-There are currently no effective therapies for diffuse malignant peritoneal mesothelioma (DMPM) patients with disease recurrence. In this study, we investigated the biology of DMPM by analyzing the EGFR family, Axl, and MET, in order to assess the presence of cross-talk between these receptors, suggesting the effectiveness of combined targeted treatments in DMPM. Method-We analyzed a series of 22 naïve epithelioid DMPM samples from a single institute, two of which showed higher-grade malignancy ("progressed"). EGFR, HER2, HER3, Axl, and MET activation and expression were investigated by biochemical analysis, real-time PCR immunofluorescence, immunohistochemistry, next-generation sequencing, miRNA, and mRNA in situ hybridization. Results-In most DMPMs, a strong EGFR activation was associated with HER2, HER3, Axl, and MET co-activation, mediated mainly by receptor heterodimerization and autocrine-paracrine loops induced by the expression of their cognate ligands. Axl expression was downregulated by miRNA34a. Mutations in MET Sema domain were exclusively found in two "progressed" DMPMs, and the combined Axl and MET inhibition reduced cellular motility in a DMPM cell line obtained from a "progressed" DMPM. Conclusion-The results indicate that the coordinated activity of multiple cross-talks between RTKs is directly involved in the biology of DMPM, suggesting the combined inhibition of PIK3 and mTOR as an effective strategy that may be easily implemented in clinical practice, and indicating that the combined inhibition of EGFR/HER2 and HER3 and of Axl and MET deserves further investigation.
目前,对于疾病复发的弥漫性恶性腹膜间皮瘤(DMPM)患者,尚无有效的治疗方法。在这项研究中,我们通过分析 EGFR 家族、Axl 和 MET,研究了 DMPM 的生物学特性,以评估这些受体之间是否存在串扰,从而评估联合靶向治疗在 DMPM 中的有效性。
我们分析了来自单个机构的 22 例初治上皮样 DMPM 样本,其中 2 例显示高级别恶性肿瘤(“进展”)。通过生化分析、实时 PCR 免疫荧光、免疫组织化学、下一代测序、miRNA 和 mRNA 原位杂交,研究了 EGFR、HER2、HER3、Axl 和 MET 的激活和表达。
在大多数 DMPM 中,强烈的 EGFR 激活与 HER2、HER3、Axl 和 MET 的共激活相关,主要由配体表达引起的受体异二聚化和自分泌-旁分泌环介导。miRNA34a 下调了 Axl 的表达。MET Sema 结构域的突变仅在两个“进展”DMPM 中发现,联合 Axl 和 MET 抑制可降低从“进展”DMPM 获得的 DMPM 细胞系的细胞迁移。
结果表明,多个 RTKs 之间的协调活性直接参与了 DMPM 的生物学特性,提示联合抑制 PI3K 和 mTOR 可能是一种有效的策略,易于在临床实践中实施,并表明联合抑制 EGFR/HER2 和 HER3 以及 Axl 和 MET 值得进一步研究。