成人型肺母细胞瘤临床病理及免疫组化

Clinicopathology and Immunohistochemistry of Adult Type Pulmonary Blastoma

目的分析成人型肺母细胞瘤的临床病理特征及免疫组化特点。方法通过光镜及免疫组织化学技术分析4例成人型肺母细胞瘤的临床病理特征及免疫组化特点。结果4例成人型肺母细胞瘤中3例为双向型肺母细胞瘤,由原始的上皮成分和原始的间叶成分共同组成;1例为上皮型肺母细胞瘤,表现为成片分化较好的分支状腺管结构,形态类似于子宫内膜样腺体;免疫组化4例细胞角蛋白广谱抗体原始上皮成分均阳性,3例波形蛋白抗体原始间质成分均阳性,2例双向型肺母细胞瘤甲状腺转移因子-1上皮成分阳性,CD1171例阳性,Ki-671例2%阳性,P53均阴性。结论成人型肺母细胞瘤是一种少见的含有类似于分化好的胎儿腺癌的原始上皮成分和原始间叶成分的双向性肿瘤,主要见于成人,具有高度的侵袭性,预后较差。

Objective To study the clinicopathologic and immunohistochemical features of adult type pulmonary blastoma （PB）. Method Four cases of adult type PB were studied with light microscopy and immunohistochemical staining. Results Three cases of PB were biphasic type and were composed of primitive epithelial component and primitive stromal component; one case of PB was epithelial type and was shown as branching glands with clustered and well-differentiated structure.Immunohistochemical studies showed that AE1/AE3 was positive in the epithelial component in four cases, Vimentin was positive in the stromal component in three cases, thyroid transrieption factor-1 was positive in two cases, CDll7 was positive in one case, Ki-67 was 2% positive in one case, while P53 was negative in all cases. Conclusions The adult type PB is a rarely seen biphasic tumor. It contains a primitive epithelial component and a primitive mesenchymal stroma, which looks like well-differentiated fetal adenocarcinoma. As highly invasive intrathoracic neoplasm, it is mainly found in adult with an unfavorable outcome.