肺淋巴管平滑肌瘤病1例并文献复习

对1例肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)的病理形态特点及免疫组织化学进行观察分析,并复习相关文献。在PLAM镜下可见肺组织较多囊腔样结构,内衬单层扁平上皮,囊壁、支气管及肺泡周围多灶性平滑肌样梭形细胞增生,正常至轻度异型,肺泡间隔、胸膜增厚。免疫组织化学示:平滑肌样梭形细胞中平滑肌肌动蛋白(smooth muscle actin,SMA)、人类黑色素瘤单克隆抗体(human melanoma black-45,HMB45)、孕激素受体(progestogen receptor,PR)均为阳性表达,雌激素受体(estrogen receptor,ER)弱阳性,囊壁衬附单层扁平上皮D2-40阳性。PLAM是一种持续进展的罕见肺部疾病,病理光学显微镜结合免疫组织化学表型可帮助确诊。

Pulmonary lymphangioleiomyomatosis: A case report and review of literature

The clinical and pathological features of a case of pulmonary lymphangioleiomyomatosis(PLAM)were analyzed and discussed in the literature.PLAM showed that the lung tissue has a lot of cystic structures and is lined with a single layer of flat epithelium.The smooth muscle-like spindle cells around the wall,bronchi and alveoli showed multifocal hyperplasia,with the thicken alveolar septum and pleural.Immunohistochemistry showed that smooth muscle actin(SMA),human melanoma black-45(HMB45),progestogen receptor(PR),estrogen receptor(ER)and D2-40 had different degrees of positive expression.PLAM is a rare and progressive lung disease.Pathological features and immunohistochemistry are important diagnostic criteria.