目的 探讨胸膜肺母细胞瘤的早期诊断及治疗。方法 回顾性分析1例由1型胸膜肺母细胞瘤逐渐进展为3型患儿的诊治过程。结果 患儿,男,生后18 d胸片未见明显异常;1岁时胸片示左肺近肺门处囊性占位,约5 cm×4 cm×4 cm,考虑为先天性肺囊性腺瘤样畸形,家长拒绝手术;3岁2个月,胸片及胸部CT提示左肺囊性实性占位,体积较前明显增大,背侧为实性,上部位巨大含气空腔;2个月后患儿出现呼吸喘憋,复查胸部CT示肿瘤完全实变,考虑胸膜肺母细胞瘤3型。手术切除后常规化疗,随访1年尚未复发。结论 1型胸膜肺母细胞瘤与先天性肺囊性腺瘤样畸形不宜鉴别,早期诊断及治疗可改善1型胸膜肺母细胞瘤的预后。
Objective To investigate the early diagnosis and treatment of pleuropulmonary blastoma in children. Methods The progress of pleuropulmonary blastoma from type 1 to type 3 was retrospectively analyzed. Results The male infant had no obvious abnormality in chest X-ray at 18 days. At one year old, X-ray film of the chest showed cystic lesions near hilum of the left lung (about 5 cm × 4 cm × 4 cm). The congenital cystic adenomatoid malformation was considered and an operation resection was suggested. However, it had been rejected by his parents. At 3 years and 2 months old, chest X-ray and CT showed left lung had a solid cystic lesion, the volume was significantly enlarged, the back side was solid and the upper part had a large gas cavity. Two months later, the patient had asthmatic suffocation. The chest CT showed the tumor was completely solid. Pleuropulmonary blastoma type 3 was considered. The patient received the conventional chemotherapy after operation excision, and was followed up for 1 year. No recurrence was observed after the treatment. Conclusions Type 1 pleuropulmonary blastoma and congenital cystic adenomatoid malformation are hard to be differentially diagnosed. Early diagnosis and treatment of type 1 pleuropulmonary blastoma can improve the prognosis.