Children peripheral neuroblastic tumor: pathology, biology and its clinical significance
10.13315/j.cnki.cjcep.2017.07.009
- VernacularTitle:儿童神经母细胞源性肿瘤的形态及分子遗传学改变及临床意义
- Author:
chun Ke XU
1
;
bin Wen GUAN
;
chun Yan XU
;
ying Xiao WANG
;
feng Li WANG
;
jie Ming ZHU
Author Information
1. 上海交通大学医学院附属新华医院病理科
- Keywords:
neuroblastoma;
ganglioneuroblastoma;
diagnostic criteria;
biology;
prognosis
- From:
Chinese Journal of Clinical and Experimental Pathology
2017;33(7):746-750
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To summarize the histological,immunophenotypic feature and MYCN (MYC gene) amplification results of peripheral neuroblastic tumours in children,and to predict its biological behavior and prognosis.Methods The histology and immunophenotype of 100 cases of neuroblastoma (NB) and ganglioneuroblastoma (GNB) were retrospectively analysed,MYCN status was detected in 60 cases.Results Among The average age of 100 cases of GN and GNB was 2.7 years old,and that of males was more than that of females.NB could be divided into three subtypes:undifferentiated,poorly differentiated and differentiating.GNB could be divided into two subtypes:intermixed (iGNB) and nodular (nGNB).Immunohistochemical staining showed neuroblastoma cells were positive for NSE,NF,PGP9.5,Syn,CgA in varying degree.Schwann cells were positive for S-100 and GFAP.MYCN amplification was detected in 1 1.67％ of the cases,and no MYCN amplification was seen in iGNB patients.Conclusion The diagnosis of peripheral neuroblastic tumours is mainly based on histological morphology,special tests (immunohistochemistry,electron microscope and cytogenetics) can helpful for identifing undifferentiated neuroblastoma cells.The prognosis of neuroblastoma derived tumors is evaluated according to the age of patients,the classification and staging of tumors,and molecular genetic alterations.
