PMID- 11921289
OWN - NLM
STAT- MEDLINE
DCOM- 20020419
LR  - 20191025
IS  - 1045-2257 (Print)
IS  - 1045-2257 (Linking)
VI  - 34
IP  - 1
DP  - 2002 May
TI  - Genetic analysis of mediastinal nonseminomatous germ cell tumors in children and 
      adolescents.
PG  - 115-25
AB  - Primary mediastinal germ cell tumors (M-GCTs) represent a heterogeneous group of 
      tumors that varies with regard to age at presentation, histologic 
      differentiation, and outcome. We retrospectively analyzed archival tissue samples 
      of mediastinal mature and immature teratomas (n = 15) and malignant 
      nonseminomatous M-GCTs (n = 20) with comparative genomic hybridization (CGH). The 
      aim of this study was to define distinct genetic subgroups of M-GCT among the 
      pediatric cohort that may differ in their clinical behavior and prognosis. All 
      pure teratomas showed normal CGH profiles. Malignant M-GCTs in infants and 
      children < 8 years old most frequently showed a gain of 1q, 3, and 20q and a loss 
      of 1p, 4q, and 6q. Gain of 12p and sex chromosomal abnormalities were not 
      observed in this age group. In contrast, the gain of 12p was the most common 
      aberration in M-GCTs that arose in children > or = 8 years old. Additional 
      recurrent changes included the loss of chromosome 13 and the gain of chromosome 
      21. All ten adolescents with malignant M-GCT were male, and five showed a gain of 
      the X chromosome. In two of these five patients, Klinefelter syndrome was 
      confirmed by cytogenetic analysis or by fluorescence in situ hybridization 
      (FISH). In conclusion, CGH analysis of M-GCTs defines distinct genetic subgroups. 
      Mediastinal teratomas show no genetic gains or losses. Malignant M-GCTs in 
      children < 8 years old show the same pattern of gains and losses identified in 
      sacrococcygeal and testicular GCTs at this age, and they lack sex-chromosomal 
      abnormalities. Malignant M-GCTs in children > or = 8 years old show the same 
      genetic profile previously reported in gonadal GCTs at this age. In addition, 
      approximately 50% demonstrate a gain of the X chromosome, consistent with 
      Klinefelter syndrome. Cooperative group studies reveal a significantly better 
      prognosis of malignant M-GCT arising in infants compared to that in adolescents, 
      suggesting that these genetic differences are associated with differences in 
      clinical behavior.
CI  - Copyright 2002 Wiley-Liss, Inc.
FAU - Schneider, Dominik T
AU  - Schneider DT
AD  - Division of Pediatric Pathology, Department of Pathology, Johns Hopkins Medical 
      Institutions, Baltimore, Maryland 21287, USA.
FAU - Schuster, Amy E
AU  - Schuster AE
FAU - Fritsch, Michael K
AU  - Fritsch MK
FAU - Calaminus, Gabriele
AU  - Calaminus G
FAU - Gobel, Ulrich
AU  - Gobel U
FAU - Harms, Dieter
AU  - Harms D
FAU - Lauer, Stephen
AU  - Lauer S
FAU - Olson, Thomas
AU  - Olson T
FAU - Perlman, Elizabeth J
AU  - Perlman EJ
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
SB  - IM
MH  - Adolescent
MH  - Allelic Imbalance
MH  - Child
MH  - Child, Preschool
MH  - Chromosome Aberrations
MH  - Female
MH  - Humans
MH  - Infant
MH  - Male
MH  - Mediastinal Neoplasms/*genetics
MH  - Neoplasms, Germ Cell and Embryonal/*genetics
MH  - Nucleic Acid Hybridization
MH  - Retrospective Studies
MH  - Seminoma/*genetics
MH  - Sex Chromosome Aberrations
MH  - Testicular Neoplasms/*genetics
EDAT- 2002/03/29 10:00
MHDA- 2002/04/20 10:01
CRDT- 2002/03/29 10:00
PHST- 2002/03/29 10:00 [pubmed]
PHST- 2002/04/20 10:01 [medline]
PHST- 2002/03/29 10:00 [entrez]
AID - 10.1002/gcc.10053 [pii]
AID - 10.1002/gcc.10053 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 2002 May;34(1):115-25. doi: 10.1002/gcc.10053.