婴幼儿先天性肺淋巴管瘤和肺血管瘤五例分析
Congenital intrapulmonary lymphangioma and pulmonary hemangioma in 5 infants and young children
目的 探讨婴幼儿先天性肺淋巴管瘤和肺血管瘤的临床、影像学及病理学表现.方法分析5例婴幼儿先天性肺淋巴管瘤及肺血管瘤的相关资料.结果 先天性肺淋巴管瘤3例、肺血管瘤2例,临床表现为咳嗽、呼吸困难、口唇紫绀、喘息等;影像学表现为局部肺野囊肿性、占位性改变,其中2例血管瘤的CT片可见血管穿入病变部位;肺组织学均表现为大小不等的被覆内皮的囊腔;免疫组化:淋巴管瘤D2-40阳性,Ⅷ因子相关抗原弱阳性;血管瘤Ⅷ因子相关抗原阳性,D2-40阴性或弱阳性;二者上皮细胞角蛋白均为阴性.结论 当遇到肺囊肿性占位性疾病时应充分考虑婴幼儿先天性肺淋巴管瘤及肺血管瘤的可能.
更多Objective To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children. Method Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with hamangioma were analyzed. Result 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. Conclusion 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.
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