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目的探讨肺低级别胎儿型腺癌(FLAC-L)的临床病理特点、免疫表型、分子遗传学改变及鉴别诊断。方法回顾性分析3例FLAC-L的临床资料、组织学形态和免疫组化标记结果,并辅以PAS/PASD染色和EGFR、K-ras基因突变检测分析。结果组织学上,肿瘤由典型的类似胎儿肺小管的腺样结构组成,瘤细胞为无纤毛柱状细胞,呈假复层排列,胞质透明或颗粒状,可见核下及核上空泡,分化好的腺腔内可见到桑葚体。免疫组化:肿瘤细胞TTF-1、CK7、β-catenin和p53弥漫(+),Syn、CgA和CD56均呈不同程度(+),napsinA呈局灶(+)。基因突变检测显示,EGFR基因18~21号外显子均无突变,K-ras基因12和13号密码子无突变,均为野生型。肿瘤细胞PAS染色(+),而PASD染色(-)。3例患者术后分别随访32、56和49个月均无复发与转移。结论 FLAC-L是一种非常少见的肺部肿瘤,低度恶性,预后好,具有独特的组织学形态。免疫组化和PAS/PASD染色有助于明确诊断。
Objective To investigate the clinicopathological features, immunophenotypes, molecular genetic changes and differential diagnosis of low-grade lung adenocarcinoma of the lung (FLAC-L). Methods The clinical data, histological features and immunohistochemical results of FLAC-L in 3 cases were retrospectively analyzed. The results of PAS / PASD staining and EGFR and K-ras mutations were analyzed. Results Histologically, the tumor consisted of the typical adenoid structure similar to the fetal pulmonary tubules. The tumor cells were cilia-free columnar cells arranged in a pseudostratified, cytoplasm transparent or granular form. The gland cavity can be seen mulberry body. Immunohistochemistry: TTF-1, CK7, β-catenin and diffuse (+) of p53, Syn, CgA and CD56 were all increased in different degrees (+) and napsinA was focal (+). Mutations in EGFR gene showed no mutation in exon 18-21 and no mutation in codons 12 and 13 of K-ras gene. All of them were wild type. Tumor cells were PAS stained (+), while PASD stained (-). Three patients were followed up for 32, 56 and 49 months respectively, with no recurrence and metastasis. Conclusion FLAC-L is a very rare pulmonary tumor, with low malignancy, good prognosis and unique histological appearance. Immunohistochemistry and PAS / PASD staining help to confirm the diagnosis.