Zhou Chun-ju, Lang Zhi-qi, Yang Jie, Wang Lin, DU Xin-yu, Liu Jing, Lu Di
Department of Pathology, Beijing Children's Hospital Affiliated to the Capital Medical University, Beijing 100045, China.
Zhonghua Er Ke Za Zhi. 2009 Oct;47(10):782-4.
To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.
Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.
All the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative.
It is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.
分析5例婴幼儿先天性肺内淋巴管瘤及血管瘤的临床、影像学及病理表现。
对3例先天性肺内淋巴管瘤及2例血管瘤患儿的资料进行分析。
5例均有咳嗽、呼吸困难、口唇发绀及气促表现。影像学检查提示部分肺野呈囊性及占位性改变。2例血管瘤患儿CT片上可见穿过血管瘤的血管。组织化学研究显示，切除组织中的囊腔大小不一，囊腔周围有一层扁平内皮组织。免疫化学研究表明，淋巴管瘤病例D2-40阳性、因子VIII弱阳性；而血管瘤病例因子VIII阳性，D2-40阴性或弱阳性，细胞角蛋白均为阴性。
通过影像学及病理检查，对于肺部有囊腔及占位性改变的患者，考虑并鉴别先天性肺内淋巴管瘤或血管瘤非常重要。
