[Fetal adenocarcinoma of the lung: a clinicopathologic analysis of six cases].
[Fetal adenocarcinoma of the lung: a clinicopathologic analysis of six cases].
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DOI：
10.3760/cma.j.issn.0529-5807.2016.09.006
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发表时间：
2016-09-08
期刊：
影响因子：
--
通讯作者：
Chen, X Y
中科院分区：
文献类型：
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作者：
Huang, H J;Chen, X Y
目的：目的：探讨胎儿肺腺癌（FACL）的临床病理特征、诊断与鉴别诊断及基因改变。方法：收集福建省立医院收治的6例FACL病例，回顾性分析其临床病理特征、免疫表型、PAS/PASD染色及表皮生长因子受体（EGFR）基因突变分析。有3名男性和3名女性患者，年龄40至75岁（中位数59岁）。肿瘤大小范围为2.5-6.0 cm（平均3.8 cm）。组织学上，肿瘤表现出典型的腺样结构，类似于胎儿肺小管。肿瘤腺体内衬无纤毛的假复层柱状上皮，胞质透明或呈颗粒状。部分细胞出现核下或核上空泡。还观察到鳞状桑椹胚。免疫组化显示肿瘤细胞TTF 1、Napsin A、CKL阳性，CKH（2/6）、突触素（1/6）、嗜铬粒蛋白A（1/6）、CD 56（2/6）和EGFR（4/6）灶性阳性。肿瘤细胞PAS/PASD染色阳性。5例EGFR突变，1例为野生型。所有6例患者均接受了手术切除，4例接受了化疗。随访1-40个月，所有患者均无复发或转移。结论：FACL是一种罕见的低恶性潜能肿瘤，具有独特的形态学特征。临床及病理上需与肺泡腺癌、腺鳞癌、成纤维细胞瘤及转移性类腺癌鉴别。FACL的主要治疗是完全手术切除和化疗，效果良好。
OBJECTIVE: To investigate the clinicopathologic features, diagnosis and differential diagnosis, and genetic alterations of fetal adenocarcinoma of lung (FACL).METHODS: Six cases of FACL were collected from Fujian Provincial Hospital, the clinicopathologic features and immunophenotype were retrospectively evaluated, PAS/PASD staining and genetic mutation analysis of epidermal growth factor receptor (EGFR) were performed.RESULTS: There were three male and three female patients, aged 40 to 75 years (median 59 years). Tumor size ranged from 2.5-6.0 cm (mean 3.8 cm). Histologically, the tumors showed classic glandular structure similar to fetal lung tubules. The neoplastic glands were lined by non-ciliated pseudostratified columnar epithelium with clear or granular cytoplasm. Some cells showed subnuclear or supranuclear vacuoles. Squamoid morules were also noted. Immunohistochemically, the tumor cells were positive for TTF1, Napsin A, CKL, and focally positive for CKH (2/6), synaptophysin (1/6), chromogranin A (1/6), CD56 (2/6), and EGFR (4/6). PAS/PASD staining was positive in the tumor cells. Five cases showed EGFR mutation, one case was of wild type. All six patients underwent surgical resection, and four had chemotherapy. All patients had no evidence of recurrence or metastasis during 1-40 months follow-up period.CONCLUSIONS: FACL is a rare tumor with low malignant potential and has distinct morphologic feature. Clinically and pathologically, it needs to be differentiated from alveolar adenocarcinoma, adenosquamous carcinoma, pneumoblastoma, and metastatic endometrioid adenocarcinoma. The primary treatment for FACL is complete surgical excision and chemotherapy, with good outcome.