Wu Bichen,Li Li,Meng Yanni,et al.Diagnosis and treatment of 8 children with pleuropulmonary blastoma presenting with respiratory symptoms as an initial manifestation[J].Journal of Clinical Pediatric Surgery,,():572-577.[doi:10.3760/cma.j.cn101785-20260312-00111]
Diagnosis and treatment of 8 children with pleuropulmonary blastoma presenting with respiratory symptoms as an initial manifestation
- Keywords:
- Pleuropulmonary Blastoma; Diagnosis; Surgical Procedures; Operative; Therapy; Treatment Outcomes
- Abstract:
- Objective To summarize the clinical characteristics,diagnostic approaches and therapeutic experiences of pediatric pleuropulmonary blastoma (PPB) and boost the understanding and early diagnostic capability of this disease. Methods A retrospective analysis was conducted for the relevant clinical data from 8 children with pathologically confirmed PPB presenting with respiratory symptoms as an initial manifestation from September 2018 to December 2025.Clinical presentations,imaging findings,pathological subtypes,treatment protocols and outcomes were examined. Results There were 4 boys and 4 girls with a median age of 38(17-59) year.Initial symptoms included cough (n=6,75.0%),fever (n=5,62.5%),tachypnea (n=4,50.0%) and chest pain (n=1,12.5%).Contrast enhanced chest CT revealed a huge cystic solid mass (n=8,100%).The lesions were left-sided (n=3) and right sided (n=5).There were atelectasis (n=7,87.5%) and pleural effusion (n=5,62.5%).Surgical procedures included lobectomy (n=5,62.5%) and tumor puncture or exploratory biopsy (n=3,37.5%).A definite diagnosis was made by surgical or biopsy specimens plus immunohistochemistry.All of them were followed up with a median period of 33 (0.5-36) month.During follow ups,there were 4 deaths.Three cases were because the families refused radical surgery and subsequent treatment while another family turned town postoperative adjuvant chemotherapy.The other 4 children survived disease free,of whom 2 received standardized postoperative chemotherapy and 2 (type I) did not. Conclusion The early clinical manifestations of pediatric PPB are non-specific and prone to misdiagnosis.Timely imaging combined with pathological,and genetic examination should be performed for suspected cases.Treatment should adopt a multidisciplinary model based on complete surgical resection,while emphasizing comprehensive patient management and family support to improve treatment compliance and overall prognosis.
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Memo
收稿日期:2026-3-12。
通讯作者:阳广贤,Email:ouyang_gx@sina.com