Childhood pleuropulmonary blastoma：a clinicopathologic study of 16 cases
10.3760/cma.j.issn.0529-5807.2014.11.007
- VernacularTitle:儿童胸膜肺母细胞瘤临床病理学观察
- Author:
Nan ZHANG
1
;
Libing FU
;
Chunju ZHOU
;
Lin WANG
;
Zhiqi LANG
;
Lejian HE
Author Information
1. 100045,首都医科大学附属北京儿童医院病理科
- Keywords:
Pleural neoplasms;
Lung neoplasms;
Child
- From:
Chinese Journal of Pathology
2014;(11):747-752
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinicopathological and immunohistochemical features , histogenesis and prognosis of pleuropulmonary blastoma ( PPB) in children.Methods PPB specimens from 16 pediatric cases with an age ranging from 1 year and 7 months to 5 years and 3 months ( mean age of 3 years) were retrieved and analyzed by routine histological , immunohistochemical and electron methods.Results Among 16 patients, there were 2 type Ⅰ, 7 type Ⅱ and 7 type Ⅲ PPB cases.Type Ⅰ PPB as multilocular cystic structure , consisted of thin fibrous wall lining the respiratory epithelium , subepithelial primitive blastema or immature mesenchymal cells , with or without rhabdomyoblastic differentiation or cartilage;Type Ⅱ PPB as cystic-solid tumor,comparing with type Ⅰ, consisted of intracystic components with appearance of anaplastic tumor cells.TypeⅢPPB consisted of completely solid mass , the same as the solid region of type Ⅱ, had mixed pattern including blastema ,undifferentiated spindle-cell proliferations and sarcomas.In addition, anaplastic tumor cells and intra-and extra-cytoplasmic eosinophilic globules were also commonly present.Epithelial components in PPB were benign.Immunohistochemical study showed primitive mesenchymal differentiation of tumors.All cases were positive for vimentin , desmin, myogenin and SMA in tumors with skeletal muscle differentiation , S-100 was positive in tumors with cartilage differentiation.All tumors were negative for synaptophysin ,CD99,and CD117.Benign epithelial components were positive for AE1/AE3 and EMA.In 12 cases, electron microscopy revealed few organelles in the primitive mesenchymal cells and rich heterochromatin in mesenchymal cells , the latter also demonstrating cytoplasmic myofilament dysplasia.Nine cases had clinical follow-up ranging from 5 to 48 months, of which 4 patients died.Conclusions PPB is a rare lung neoplasm of children under the age of 6 years,with distinct pathological morphology .PPB may arise from lung or pleura mesenchymal cells and has a poor clinical outcome.
