Childhood pleuropulmonary blastoma: a clinicopathologic study of 16 cases

• Published in: Zhonghua bing li xue za zhi Vol. 43; no. 11; p. 747
• Main Authors: Zhang, Nan, Fu, Libing, Zhou, Chunju, Wang, Lin, Lang, Zhiqi, He, Lejian
• Format: Journal Article
• Language: Chinese
• Published: China 01.11.2014
• Subjects: Child, Preschool; Cysts - pathology; Desmin - analysis; Female; Humans; Infant; Lung Neoplasms - chemistry; Lung Neoplasms - pathology; Male; Microscopy, Electron; Myogenin - analysis; Prognosis; Pulmonary Blastoma - chemistry; Pulmonary Blastoma - pathology; Sarcoma - pathology; Vimentin - analysis
• ISSN: 0529-5807
• DOI: 10.3760/cma.j.issn.0529-5807.2014.11.007

To study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children. 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. 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. Type III PPB 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, anaplast