肺淋巴管平滑肌瘤病临床病理特征观察及文献复习

Pulmonary lymphangioleiomyomatosis:a clinicopathological study and review of the literature

目的 探讨肺淋巴管平滑肌瘤病 (pulmonarylymphangioleiomyomatosis,PLAM )组织学、免疫组化及预后特点。 方法 对 2例PLAM经支气管肺活检组织 ,进行组织学观察及免疫组化检测 ,并复习文献进行研究。结果 2例PLAM均为育龄期女性。本病具有一定的形态学和免疫组化特征 ,肺淋巴管、血管和小气道周围异常平滑肌细胞弥漫性增生 ,分两种类型 :囊肿为主型和平滑肌为主型。 2例组织形态学相似 ,异常平滑肌细胞呈梭形、多边形及上皮样 ,核大 ,不规则多形性 ,可见核仁和核内包涵体 ,未见核分裂象。免疫组化梭形、多边形及上皮样细胞表达HMB4 5、SMA、actin和ER、PR阳性。结论 PLAM是罕见的肺慢性进行性恶化的肿瘤性疾病。PLAM病理组织学评分 (LAMhistologicscore,LHS)是评价PLAM预后的指标。依据临床和组织学特点 。

Purpose To report 2 cases of pulmonary lymphangioleiomyomatosis (PLAM) and to discuss its histological, immunohistochemical features and prognosis. Methods Transbronchial lung biopsies were performed, and histology and immunohistochemistry (IHC) were used in 2 cases, with review of the literature. Results It was found in women of childbearing age in 2 cases. It had its morphological and immunohistochemical characteristics by peribrochical, perivascular and perilymphatic proliferation of abnormal smooth muscle cells. Histologically, two types of pulmonary lesions were observed,ie, a predominantly cystic type and a predominantly muscular type. Morphologically,two cases were similar; which was composed of spindle, polygonal and epitheliod cells with oxyphilic cytoplasm, significant nuclear atypia with enlarged, irregular nuclei, nucleoli and intranuclear pseudo-inclusions. Mitostic figures were not found. IHC showed that characteristic smooth muscle cells were positive for SMA, HMB45, actin, estrogen and progesterone recepter. Conclusions PLAM is a rare neoplastic proliferative disease in the lung. PLAM histologic score(LHS) may provide a basis for determining the prognosis of PLAM. The diagnosis can be made by evaluation of the clinical characteristics, histological and immunohistochemical features.