Clinicopathologic features and prognosis of 28 cases of pleuralpulmonary blastoma in children
10.3760/cma.j.cn112152-20190316-00165
- VernacularTitle:28例小儿胸膜肺母细胞瘤的临床病理特征及预后
- Author:
Fenghua WANG
1
;
Jianhua LIANG
;
Le LI
;
Jue TANG
;
Hui WANG
;
Jiahang ZENG
;
Wei LIU
Author Information
1. 广州市妇女儿童医疗中心胸外科 510120
- Keywords:
Pleuropulmonary blastoma;
Children;
Lung neoplasms;
Clinicopathologic feature;
Prognosis
- From:
Chinese Journal of Oncology
2020;42(7):575-579
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical characteristics, therapeutic methods and prognosis of pleuropulmonary blastoma in children.Methods:The clinical data of 28 patients with pleuropulmonary blastoma diagnosed in Guangzhou Women and Children′s Medical Centre from November 2008 to May 2018 were collected and retrospectively analyzed.Results:Of the 28 patients, 18 were male and 10 were female, aged from 22 days to 5 years 10 month, the average age was 2 years 6 months. One patient underwent biopsy and other 27 underwent operation, 14 patients with type Ⅱ/Ⅲ pleuropulmonary blastoma received postoperative chemotherapy. Five patients were pathologically diagnosed as typeⅠpleuropulmonary blastoma, 5 were type Ⅱ pleuropulmonary blastoma and 18 were type Ⅲ pleuropulmonary blastoma. During the follow-up period of 24 patients, 15 patients were disease free survival, 3 patients relapsed within 6 months, 10 months and 18 months after chemotherapy, respectively. One patient who received postoperative chemotherapy suffered a bone metastasis within 11 months, 2 patient without chemotherapy relapsed within 2 months and suffered bone or renal metastasis within 3 months, respectively. Three patients who left hospital voluntarily died within 1 month.Conclusions:Pleuropulmonary blastoma is a highly malignant and rapidly progressed neoplasm. Patients with type Ⅰ pleuropulmonary blastoma have good prognoses while the prognoses of Ⅱ/Ⅲ pleuropulmonary blastoma are poor. Postoperative chemotherapy seems to improve the survival of patients withⅡ/Ⅲ pleuropulmonary blastoma.