Analysis of 8 cases of primary mediastinal large B-cell lymphoma
10.3760/cma.j.cn112140-20241104-00788
- VernacularTitle:儿童原发纵隔大B细胞淋巴瘤8例分析
- Author:
Linlin ZHANG
1
;
Chunju ZHOU
;
Nan LI
;
Shuang HUANG
;
Meng ZHANG
;
Ling JIN
;
Jing YANG
;
Yonghong ZHANG
;
Yanlong DUAN
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科 儿童血液病与肿瘤分子分型北京市重点实验室 儿童肿瘤国家临床重点专科 儿科重大疾病研究教育部重点实验室，北京100045
- Publication Type:Journal Article
- Keywords:
Child;
Lymphoma, large B-cell, diffuse;
Antineoplastic combined chemotherapy protocols
- From:
Chinese Journal of Pediatrics
2025;63(8):901-905
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, pathologic characteristics, treatment and prognosis of primary mediastinal large B-cell lymphoma (PMBCL) in children.Methods:Clinical data including clinical manifestations, treatment, clinical efficacy of 8 cases of childhood PMBCL treated in Beijing Children′s Hospital, Capital Medical University from March 2017 to February 2024 were collected retrospectively, the clinical characteristics and prognosis of them were summarized.Results:Among the 8 children, there were 5 males and 3 females. The age at the time of initial diagnosis was 11.0 (10.3, 13.5) years. The first clinical symptoms were cough (8 cases) and stridor (6 cases). The lesions most often involved the mediastinum (8 cases), lungs (5 cases, hilum more often), pericardium (5 cases), and pleura (4 cases). Extra thoracic invasion was present in 4 cases, 7 cases had huge tumor lesions and 7 cases were phase Ⅲ clinical stage. Except for 1 case who underwent surgical resection of the tumor, the remaining 7 cases were treated with DA-EPOCH+R (dose adjusted-etoposide+prednisone+vincristine+cyclo-phosphamide+doxorubicin+rituximab) chemotherapy. The follow-up time was 25.0 (10.5, 43.3) months, with 7 cases in complete and partial metabolism response, 1 case had disease progression. All 8 cases survived.Conclusions:PMBCL is most common in school-age boys and most of them present with huge mediastinal tumor focus. PMBCL expresses B-cell spectrum antigens and weakly expresses CD30.The application of DA-EPOCH+R is effective in the treatment of PMBCL in children.
