Wang Liang, Lin Yongbin, Cai Qingqing, Long Hao, Zhang Yu, Rong Tiehua, Ma Guowei, Liang Ying
1 Department of Hematologic Oncology, Sun Yat-sen University Cancer Center; State Key Laboratory of Oncology in South China ; Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, China ; 2 Department of Thoracic Surgery, Sun Yat-sen University Cancer Center; State Key Laboratory of Oncology in South China ; Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, China ; 3 Department of Medical Oncology, Sun Yat-sen University Cancer Center; State Key Laboratory of Oncology in South China ; Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, China ; 4 Department of Pathology, Sun Yat-sen University Cancer Center; State Key Laboratory of Oncology in South China ; Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, China.
J Thorac Dis. 2015 Sep;7(9):1556-62. doi: 10.3978/j.issn.2072-1439.2015.05.11.
Primary pulmonary lymphoepithelioma-like carcinoma (LELC) is a distinct rare subtype of lung cancer. The prevalence of anaplastic lymphoma kinase (ALK) rearrangement and epidermal growth factor receptor (EGFR) mutation in primary pulmonary LELC had not been thoroughly investigated.
We investigated a cohort of 42 patients with primary pulmonary LELC and genotyped for ALK rearrangement and EGFR mutation. ALK rearrangement was detected by fluorescence in situ hybridization (FISH). EGFR mutational analysis of exons 18 through 21 was analyzed by TaqMan real-time polymerase chain reaction (PCR).
Epstein-Barr virus-encoded RNAs (EBERs) showed positive signals in all 42 patients. By immunohistochemistry staining, all patients demonstrated positive expression of CK5/6 and P63, but almost all patients were negative for TTF-1 (34/34, 100%) or CK7 (34/35, 97.1%). None of the 42 patients had ALK rearrangement. Of 42 patients tested, only one patient (2.4%) harbored L858R mutation and gefitinib was applied to this case, however no objective response was observed and the progression free survival (PFS) time was only 1 month.
Primary pulmonary LELC is a unique histological subtype of lung cancer. ALK rearrangement and EGFR mutation are lack and they may not be the oncogenic driver gene in pulmonary LELC. Future efforts should be made to explore other oncogenic driver gene to guide targeted therapy in this rare disease to determine the optimal treatment.
原发性肺淋巴上皮瘤样癌（LELC）是一种独特的罕见肺癌亚型。原发性肺LELC中间变性淋巴瘤激酶（ALK）重排和表皮生长因子受体（EGFR）突变的发生率尚未得到充分研究。
我们调查了42例原发性肺LELC患者，并对其进行ALK重排和EGFR突变基因分型。通过荧光原位杂交（FISH）检测ALK重排。通过TaqMan实时聚合酶链反应（PCR）分析外显子18至21的EGFR突变。
爱泼斯坦-巴尔病毒编码的RNA（EBERs）在所有42例患者中均显示阳性信号。通过免疫组织化学染色，所有患者均表现出CK5/6和P63的阳性表达，但几乎所有患者的TTF-1（34/34，100%）或CK7（34/35，97.1%）为阴性。42例患者均无ALK重排。在42例检测患者中，只有1例患者（2.4%）存在L858R突变，并对该病例应用了吉非替尼，然而未观察到客观反应，无进展生存期（PFS）仅为1个月。
原发性肺LELC是一种独特的肺癌组织学亚型。ALK重排和EGFR突变缺乏，它们可能不是肺LELC的致癌驱动基因。未来应努力探索其他致癌驱动基因，以指导这种罕见疾病的靶向治疗，确定最佳治疗方案。
