Zhang Xu,Zeng Qi,Zhang Na,et al.Diagnosis and treatment of pediatric pleuropulmonary blastoma: a report of 38 cases[J].Journal of Clinical Pediatric Surgery,,19():63-68.[doi:10.3969/j.issn.1671-6353.2020.01.013]
Diagnosis and treatment of pediatric pleuropulmonary blastoma: a report of 38 cases
- Keywords:
- Pleuropulmonary Blastoma; Surgical Procedures; Operative; Treatment; Child
- CLC:
- R726;R734.3
- Abstract:
- Objective To explore the onset age,clinical manifestations,pathological classifications and characteristics,treatment principles and outcomes of pediatric pleuropulmonary blastoma (PPB).Methods Retrospective analysis was conducted for clinical data of 38 PPB children,including age of onset,clinical manifestations,familial disease characteristics,pathological types,treatment and prognosis from March 2002 to March 2018.Results There were 18 boys and 20 girls.The non-neoplastic lesion of initial diagnosis was 47.4%.The intervention time from symptom onset to surgery/chemotherapy was 5 to 210 days.For typeⅠ(n=4,10.5%),the median diagnostic age was 22 months; for type Ⅱ(n=12,31.6%),the median diagnostic age 37 months; for type Ⅲ (n=22,57.9%),the median diagnostic age 39.5 months.The treatments included surgery and chemotherapy.During operation,the maximal diameter of tumor was 2-18 cm,operative duration 25-210 min and intraoperative blood loss 2-500 ml.For one case of pleural exfoliation,the volume of intraoperative bleeding was 500 ml.The chemotherapeutic regimens of IVADo (ifosfamide+vincristine+actinomycin D+doxorubicin)and IVA (ifosfamide+vincristine+actinomycin D)were prescribed.The 5-year disease-free survival rate was 69.2%.Conclusion PPB is a rare form of malignant pediatric tumor.Due to non-specific clinical manifestations,it has a high misdiagnostic rate and a poor prognosis.A combination of surgery and chemotherapy is required for its treatment.Pediatricians should understand its severity and stay on a high alert for pneumothorax,multiple cystic lung lesions,a history of lung cysts,a family history of PPB and chest space-occupying lesions of DICER1 familial syndrome.Close follow-ups are recommended.
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Memo
收稿日期:2018-05-30。
通讯作者:曾骐,Email:zengqi-1@163.com