肺原发性肝样腺癌临床病理特征研究

Clinicopathologic characteristics of primary hepatoid adenocarcinoma of the lung

目的分析肺肝样腺癌(HAL)的临床病理特征、诊断、免疫表型、基因检测及治疗预后等。方法对1例HAL临床及影像学、组织学形态、免疫组化及基因检测结果等进行观察,并结合相关文献综合分析。结果患者为48岁吸烟男性,镜下肿瘤具有肝细胞样和腺样分化特征,血清AFP升高。免疫组化:Hepatocyte,AFP, Arginase-1均阳性,ARMS-PCR法均未检测到EGFR,ALK/ROS1,KRAS及BRAF突变。结合相关文献分析:HAL常见于有吸烟史的男性,血清AFP值升高也是该肿瘤的一个特点。肿物多见于肺上叶,体积较大,易发生淋巴结和远处转移,预后相对较差。结论 HAL非常少见,易误诊,其诊断需结合形态学特点、临床病理特征及免疫组化结果等。

Objective To explore the clinicopathologic characteristics,diagnosis,immunophenotype,gene detection and prognosis of primary hepatoid adenocarcinoma of the lung (HAL). Methods A case of hepatoid adenocarcinoma of the lung was analyzed with clinical manifestations,histology,immunohistochemical staining and gene detection,and relevant literatures were reviewed. Results The patient was a 48-years-old man with smoking history. Microscopically,the tumor has the characteristics of hepatocellular carcinoma and adenoid differentiation,also serum AFP was elevated. The immunohistochemical results showed that Hepatocyte,AFP and Arginase-1were positive. No mutations were detected for EGFR,ALK/ROS1,KRAS and BRAF by ARMS-PCR. Combining with literature analysis,HAL is common in males and most patients with this tumor are smokers. Serum AFP in very high levels has been a distinguishing feature of this tumor. HAL usually presents as a large bulky solitary mass in the upper lobe. Lymph nodes and distant metastases are prone to occur. Therefore,the prognosis is very poor. Conclusion HAL is a rare malignant tumor and easy to be misdiagnosed. The diagnosis of primary hepatoid adenocarcinoma of the lung should be combined with morphological features,clinicopathological features and immunohistochemical findings.