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Advances
in the diagnosis, molecular genetics, and treatment of pediatric embryonal CNS
tumors
MacDonald
TJ, Rood BR, Santi MR, Vezina G, Bingaman K, Cogen PH, Packer RJ
Departments
of Hematology/Oncology, Pathology, Radiology, Neurosurgery, and Neurology,
Children's Hospital National Medical Center, Washington, DC, USA
Embryonal
central nervous system (CNS) tumors are the most common group of malignant brain
tumors in children.
The diagnosis and classification of tumors belonging to this family have been
controversial; however, utilization of molecular genetics is helping to refine
traditional histopathologic and clinical classification schemes.
Currently, this group of tumors includes medulloblastomas, supratentorial
primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors,
ependymoblastomas, and medulloepitheliomas.
While the survival of older children with nonmetastatic medulloblastomas has
improved considerably within the past two decades, the outcomes for infants and
for those with metastatic medulloblastomas or other high-risk embryonal CNS
tumors remain poor.
It is anticipated that the emerging field of molecular biology will greatly aid
in the future stratification and therapy for pediatric patients with malignant
embryonal tumors.
In this review, recent advances in the diagnosis, molecular genetics, and
treatment of the most common pediatric embryonal CNS tumors are discussed.
PMID: 12697942 [PubMed - in process]
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