A prognostic analysis of pediatrics central nervous system small cell tumors: evaluation of EGFR family gene amplification and overexpression.

Weidong Liu,Shigang Zhang,Qi Pang,Qingke Cui,Jiyue Wang,Ting Gui,Liyong Zhang

doi:10.1186/1746-1596-9-132

Weidong Liu, Shigang Zhang + Show 5 more

Open Access

https://doi.org/10.1186/1746-1596-9-132

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Abstract

BackgroundCentral nervous system (CNS) tumors are the most common solid tumors that occur in children, however there were few big-data follow-up analysis published in China. Overexpression of epidermal growth factor receptor (EGFR) family members was reported on glioblastoma (GBM) and medulloblastoma (MB) before. However, the correlation between EGFR family members expression with prognosis of MB, supratentorial primitive neuroectodermal tumor (PNET) and small cell GBM is unclear in Chinese children.MethodsA retrospective and survival analysis was performed on children (age ≤ 16 years) diagnosed as CNS primary small cell tumors in the Affiliated Provincial Hospital, Shandong University from 2000 to 2012, including MB (n = 44), PNET (n = 8) and small cell GBM (n = 19). The expression of EGFR, ERBB-2, ERBB-3 and ERBB-4 were detected by immunohistochemistry (IHC). The fluorescence in situ hybridization (FISH) was used to observe the amplification of EGFR and ERBB-2 gene.ResultsMedian survival times of MBs, small GBMs and PNETs were 23 ± 6.7 months, 8 ± 4.7 months and 10 ± 1.4 months. Expression and amplification of ERBB-2, ERBB-3 and ERBB-4 were not observed in all tumor samples. The multiply Cox regression suggested the overexpression and amplification of EGFR were negative prognostic factors for MB. Radiotherapy had the positive function for all pediatric patients.ConclusionOverexpression of EGFR predicts poor outcomes of MBs, small cell GBMs and PNETs, suggesting those three CNS tumor subtypes can be considered as one group for the potential common mechanism. The current individual treatment and big data analysis of pediatric CNS embryonal tumors and GBM continues to be very challenging in China.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7649640001237474

Highlights

Central nervous system (CNS) tumors are the most common solid tumors that occur in children, there were few big-data follow-up analysis published in China
The clinical features and survival times of pediatrics CNS small cell tumors From January 2000 to December 2012, 71 cases pediatric (≤16 year-old) CNS primary small cell tumors were followed up including MB (44 cases), small cell GBM
At the end of December 31, 2012, 65 patients were interviewed, of which 40 MB patients, 17 cases small cell GBM and 8 cases primitive neuroectodermal tumor (PNET), accounted for 91.55% of total small cell tumors. 45 patients were dead at the endpoint of observation

Summary

Introduction

Central nervous system (CNS) tumors are the most common solid tumors that occur in children, there were few big-data follow-up analysis published in China. Central nervous system (CNS) tumors are the second most common group of malignancies among children; leukemias as a group are the most common. The overall average annual incidence rate for pediatric CNS tumors (ages 0–19 years) is 5.26 per 100,000 [1]. Embryonal tumors are the most common CNS neoplasms in infants less than 36 months of age. In USA (United States of America), the incident rate of CNS embryonal tumors under 14 years old is 0.8 per 100,000 and the median age is 9. There were few reports related with the large-scale follow-up or prognostic analysis of pediatrics CNS embryonal tumors in china

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Conclusion