pediatric germ cell tumors of the central nervous system

Intracranial pediatric germ cell tumors (IPGCTs) are rare and heterogeneous neoplasms and vary in histological differentiation, prognosis and clinical behavior.

Germinoma and mature teratoma are GCTs that have a good prognosis, while other types of GCTs, termed nongerminomatous malignant germ cell tumors (NGMGCTs), are tumors with an intermediate or poor prognosis.

miRNA

There is a miRNA profile of pediatric primary intracranial GCTs. (20178649)

Most of the differentially expressed miRNAs were downregulated in germinomas, but miR-142-5p and miR-146a were upregulated. (20178649)

Expression profile

There is a distinct mRNA profile correlating with GCT histological differentiation and prognosis. (20178649)

Genes responsible for self-renewal (such as POU5F1 (OCT4), NANOG and KLF4) and the immune response were abundant in germinomas, while genes associated with neuron differentiation, Wnt/beta-catenin pathway, invasiveness and epithelial-mesenchymal transition (including SNAI2 (SLUG) and TWIST2) were abundant in NGMGCTs. (20178649)

Clear transcriptome segregation based on patient survival is observed, with malignant NGMGCTs being closest to embryonic stem cells. (20178649)

CGH

Chromosome copy number variations (CNVs) at cytobands 4q13.3-4q28.3 and 9p11.2-9q13 correlated with GCT malignancy and clinical risk. (20178649)

Six genes (BANK1, CXCL9, CXCL11, DDIT4L, ELOVL6 and HERC5) within 4q13.3-4q28.3 were more abundant in germinomas.(20178649)

See also

 pediatric germ cell tumors (PGCTs)

References

 Pediatric primary central nervous system germ cell tumors of different prognosis groups show characteristic miRNome traits and chromosome copy number variations. Wang HW, Wu YH, Hsieh JY, Liang ML, Chao ME, Liu DJ, Hsu MT, Wong TT. BMC Genomics. 2010 Feb 24;11:132. PMID: 20178649 [Free]