Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China.
Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China.
J Pediatr Surg. 2020 Jul;55(7):1351-1355. doi: 10.1016/j.jpedsurg.2019.06.009. Epub 2019 Jun 27.
This study was performed to investigate the age at onset, clinical manifestations, pathological types and features, treatment, and prognosis of pleuropulmonary blastoma (PPB) in children in an attempt to reduce the misdiagnosis rate and achieve early detection and timely intervention.
We retrospectively studied the clinical data of 41 pediatric patients with PPB who were treated in our center from March 2002 to November 2018. The data comprised the age at onset, clinical manifestations, characteristics of familial diseases, pathological types, surgical procedures, and prognosis.
Twenty male and 21 female patients were included, with a 0.95:1.00 male:female ratio. In total, 51.2% of the patients were misdiagnosed as having nonneoplastic lesions at the first presentation. The interval from symptom onset to surgery/chemotherapy ranged from 5 to 210 days. The pathological types were type I (cystic) PPB (n = 5, 11.9%), for which the median age at diagnosis was 21 months (range, 8-24 months); (solid/cystic) II PPB (n = 12, 28.6%), for which the median age at diagnosis was 37 months (range, 22-112 months); and type III (solid) PPB (n = 23, 54.8%), for which the median age at diagnosis was 39 months (range, 19-156 months). The pathologic type was undefined in one patient (2.4%). The patients were mainly treated by surgery and chemotherapy. The 5-year disease-free survival rate was 69.2%.
The clinical manifestations of PPB are nonspecific, its misdiagnosis rate is high, and it has a poor prognosis. Pediatricians should be aware of the seriousness of PPB. The possibility of PPB should be considered in children with pneumothorax, multiple pulmonary cystic lesions, a family history of pulmonary cysts, a family history of PPB, or space-occupying lesions associated with DICER1 syndromes. The lesion should be closely monitored and surgically removed if necessary. The nature of the lesion should be identified early to minimize the risk of progression of the PPB to worse types because of misdiagnosis and missed diagnosis. Multidisciplinary treatment including surgery, chemotherapy, and/or radiotherapy can be applied to patients with PPB.
Treatment study.
Level III.
本研究旨在探讨儿童胸膜肺胚细胞瘤（PPB）的发病年龄、临床表现、病理类型及特征、治疗及预后，以期降低误诊率，实现早期发现和及时干预。
回顾性分析 2002 年 3 月至 2018 年 11 月在我院治疗的 41 例儿童 PPB 患者的临床资料，内容包括发病年龄、临床表现、家族性疾病特征、病理类型、手术方式及预后。
男 20 例，女 21 例，男女比例为 0.95:1.00。首次就诊时，51.2%的患者误诊为非肿瘤性病变。从症状出现到手术/化疗的时间间隔为 5～210 d。病理类型：Ⅰ型（囊性）PPB（n=5，11.9%），中位诊断年龄为 21 个月（8～24 个月）；（实性/囊性）Ⅱ型（n=12，28.6%），中位诊断年龄为 37 个月（22～112 个月）；Ⅲ型（实性）PPB（n=23，54.8%），中位诊断年龄为 39 个月（19～156 个月）。1 例患者（2.4%）病理类型不明确。患者主要接受手术和化疗治疗。5 年无病生存率为 69.2%。
PPB 的临床表现不具特异性，误诊率高，预后差。儿科医生应认识到 PPB 的严重性。对于气胸、多发肺囊性病变、肺囊肿家族史、PPB 家族史或伴有 DICER1 综合征的占位性病变的患儿，应考虑到发生 PPB 的可能性。如果有必要，应密切监测病变并进行手术切除。应尽早明确病变性质，避免因误诊和漏诊导致 PPB 向更严重类型进展的风险增加。对于 PPB 患者可采用包括手术、化疗和/或放疗在内的多学科治疗。
治疗研究
III 级
