Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia. ASST Spedali Civili, Brescia, Italy.
Medical Oncology Unit, ASST Bergamo Ovest, Treviglio, Italy.
Lung Cancer. 2023 May;179:107176. doi: 10.1016/j.lungcan.2023.107176. Epub 2023 Mar 22.
Enteric-type adenocarcinoma of the lung (lung-ETAC, former pulmonary enteric adenocarcinoma, PEAC) is a rare subtype of non-small cell lung cancer (NSCLC), which shares morphological and immunohistochemical features with lung and colorectal adenocarcinoma. Few data are available on patient prognosis, possible prognostic factors and systemic approach to metastatic disease. We performed a pooled analysis and a systematic review of published lung-ETAC, along with an additional case description. Thirty-one eligible publications were identified, providing data from 126 patients. In the 127 patients overall analyzed, median overall survival (OS) was 56.0 (range 36.7-75.3) months in early-stage patients and 14.0 (range 4.5-23.5) months in those with advanced/metastatic disease. Median disease-free survival (DFS) after radical surgery was 24 (range 22.6-35.1) months. Smoking status (HR 4.304, 95% CI: 1.261-14.693, p = 0.020) and node involvement (HR 1.853, 95% CI: 1.179-2.911, p = 0.007) were the negative independent prognostic factors at multivariate analysis. As regards systemic therapies for advanced cases, no firm conclusions were drawn about the efficacy of lung cancer-oriented chemotherapy regimens as opposed to colon cancer-oriented ones. Molecular analysis of lung-ETAC revealed a relatively high mutational rate, with alterations in several druggable molecular pathways, KRAS and NRAS (31%) were the most frequently mutated oncogenes, followed by ROS1 (15%), RET (13%), BRAF (11%), EGFR (8%) and ALK (6%). Moreover, 3 (15%) out of 20 cases showed DNA mismatch repair deficiency (dMMR). In conclusion, advanced lung-ETAC patients appeared to have a better prognosis compared to other subtypes of NSCLC. Moreover, the mutational rate and microsatellite instability found in lung-ETACs suggest that a significant proportion of these patients could benefit from target therapies and immunotherapy.
肺肠型腺癌（lung-ETAC，前称肺肠型腺癌，PEAC）是一种罕见的非小细胞肺癌（NSCLC）亚型，其形态学和免疫组织化学特征与肺和结直肠腺癌相似。关于患者预后、可能的预后因素以及转移性疾病的全身治疗方法的数据很少。我们对已发表的 lung-ETAC 文献进行了汇总分析和系统评价，并附加了一个病例描述。确定了 31 篇符合条件的文献，提供了 126 名患者的数据。在总共分析的 127 名患者中，早期患者的中位总生存期（OS）为 56.0（范围 36.7-75.3）个月，晚期/转移性疾病患者为 14.0（范围 4.5-23.5）个月。根治性手术后的中位无病生存期（DFS）为 24（范围 22.6-35.1）个月。多因素分析显示，吸烟状态（HR 4.304，95%CI：1.261-14.693，p=0.020）和淋巴结受累（HR 1.853，95%CI：1.179-2.911，p=0.007）是独立的负预后因素。关于晚期病例的全身治疗，尚无法确定针对肺癌的化疗方案与针对结肠癌的化疗方案相比的疗效。对 lung-ETAC 的分子分析显示，其突变率相对较高，存在多个可靶向的分子途径改变，KRAS 和 NRAS（31%）是最常突变的癌基因，其次是 ROS1（15%）、RET（13%）、BRAF（11%）、EGFR（8%）和 ALK（6%）。此外，20 例中有 3 例（15%）表现出 DNA 错配修复缺陷（dMMR）。总之，与其他 NSCLC 亚型相比，晚期 lung-ETAC 患者的预后似乎更好。此外，在 lung-ETAC 中发现的突变率和微卫星不稳定性表明，相当一部分患者可能受益于靶向治疗和免疫治疗。
