摘要目的 探讨肺毛细血管瘤病的临床病理特征,提高对其临床及病理改变的认识.方法 对2例肺毛细血管瘤病的临床资料和组织学形态进行回顾性分析,并采用组织化学染色和免疫组织化学染色(EnVision法)研究其形态和免疫表型特点.结果 患者主要临床表现为喘憋,影像学(CT及肺动脉造影)特点为双肺多发结节影,肺动脉增宽,组织学表现为灶状分布的肺问质和肺泡壁毛细血管增生,小动脉肌性化,免疫组织化学染色提示病灶中肥大细胞数量增多,血小板衍生生长因子受体(PDGFR)-β表达上调,Ki-67阳性指数小于1％,p53阴性.结论 肺毛细血管瘤病是一种肺毛细血管增生性疾病,其发病机制可能与肥大细胞数量和PDGFR-β上调有关.病理医师在肺毛细血管瘤病的正确诊断和治疗中起重要作用,应提高对该病的认识.

abstractsObjective To investigate the clinicopathologic features of pulmonary capillary hemangiomatosis(PCH).Methods The clinical and pathologic profiles of 2 PCH cases were evaluated.Immunohistochemical study ( EnVision method) was performed on fixed tissues.The biologic behavior was analyzed with follow-up data.Results The main presenting symptom was dyspnea.Chest radiography of the two cases depicted diffuse,ground-glass nodules,accompanied by enlarged central pulmonary arteries.Microscopically,the most distinctive feature was proliferation of capillary channels within pulmonary interstitium and alveolar walls,accompanied by muscularization of arterioles.Immunohistochemical study showed an abundance of mast cells in the lesion,and staining for platelet-derived growth factor receptor-beta (PDGFR-β) localized to vascular smooth muscles surrounding the proliferating capillaries and the mast cells.The index of Ki-67 was less than 1 percent and the p53 was negative.Conclusions PCH is a rare vascular proliferative disease of yang patients. Increased number of mast cell and the up-regulation of PDGFR-β may suggest mechanism for PCH.The clinical and radiologic diagnosis of PCH can be very difficult,and the histological examination is regarded as the most reliable means to establish the diagnosis.Pathologists should improve their knowledge on PCH.