小儿胸膜肺母细胞瘤的诊治分析

目的探讨小儿胸膜肺母细胞瘤（PPB）的临床及病理学特征和诊治方法。方法对1例2岁4个月女孩突发呼吸困难7天,经抗感染无效,X线片、CT检查呈左肺野巨大圆形软组织肿块,行开胸肿瘤活检,一周后予以介入治疗。结果手术见肿瘤占据整个左侧胸腔,肺,累及纵隔及胸膜,不能手术切除,仅取活检。病理学常规检查：组织结构见分化较差的小圆细胞、梭形细胞,间质成分含不成熟软骨小岛,未见腺管分化;免疫组织化学检查：Vimentin阳性,明确诊断为PPB。患儿经肿瘤血管灌注顺铂及超液化碘油及栓塞介入手术后两天死亡。结论小儿胸膜肺母细胞瘤是罕见胚胎性恶性肿瘤,恶性程度高,预后极差。病理及免疫组织化学检查有助于诊断。早期诊断有助于临床治疗,改善预后。

Analysis of diagnosis and treatment of pleuropulmonary blastoma in children

Objective To explore the characteristics in clinic and pathology of pleuropulmonary blastoma in children.Method A two years and four months old girl with paroximal dyspnea for 7 days.There was no regression treated with antibiotics.X-ray,CT on thorax showed huge spherical mass with soft tissue density in left side.The interventional therapy performed one week later.Result Operation showed that the whole left thorax,lung were occupied by the tumor while the pleura of mediastina and parietal pleura invaded.The tumor can not be resected so biopsy was performed.The tissue structures showed unfavourable differentiation small round cell and spindle cell and foci of immature cartilage were also noted with no glandular formation.The immunohistochemistry stain showed Vimentin positive.The girl died after two days undertaken artery perfusion with cisplatin through tumor＇s nourishing artery followed embolization.Conclusion PPB of children is rare embryological malignant tumor with poor prognosis.It is helpful to diagnosis and treatment by routine pathological examination and immunohistochemistry.