This Article Full Text Full Text (PDF) CME: Take the course for this article: Advances in the Diagnosis, Molecular Genetics, and Treatment of Pediatric E... eLetters: Submit a response to this article Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services E-mail this article link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Reprints/Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by MacDonald, T. J. Articles by Packer, R. J. Search for Related Content PubMed PubMed Citation Articles by MacDonald, T. J. Articles by Packer, R. J.

Advances in the Diagnosis, Molecular Genetics, and Treatment of Pediatric Embryonal CNS Tumors

^a Departments of Hematology/Oncology, ^b Pathology, ^c Radiology, ^d Neurosurgery, and ^e Neurology, Children’s Hospital National Medical Center, Washington, DC, USA

Correspondence: Tobey J. MacDonald, M.D., Children’s Hospital National Medical Center, Department of Hematology/Oncology, 111 Michigan Avenue, NW, Washington, DC 20010, USA. Telephone: 202-884-2800; Fax: 202-884-5685; e-mail: tmacdona{at}cnmc.org

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.

Key Words. Primitive neuroectodermal tumor • Medulloblastoma • Atypical teratoid/rhabdoid tumor • Diagnosis • Molecular genetics • Treatment

This article has been cited by other articles:

				R. Vibhakar, G. Foltz, J.-g. Yoon, L. Field, H. Lee, G.-y. Ryu, J. Pierson, B. Davidson, and A. Madan Dickkopf-1 is an epigenetically silenced candidate tumor suppressor gene in medulloblastoma Neuro-oncol, April 1, 2007; 9(2): 135 - 144. [Abstract] [Full Text] [PDF]

				R. J. Packer, A. Gajjar, G. Vezina, L. Rorke-Adams, P. C. Burger, P. L. Robertson, L. Bayer, D. LaFond, B. R. Donahue, M. H. Marymont, et al. Phase III Study of Craniospinal Radiation Therapy Followed by Adjuvant Chemotherapy for Newly Diagnosed Average-Risk Medulloblastoma J. Clin. Oncol., September 1, 2006; 24(25): 4202 - 4208. [Abstract] [Full Text] [PDF]

				M. Tsikitis, Z. Zhang, W. Edelman, D. Zagzag, and G. V. Kalpana Genetic ablation of Cyclin D1 abrogates genesis of rhabdoid tumors resulting from Ini1 loss PNAS, August 23, 2005; 102(34): 12129 - 12134. [Abstract] [Full Text] [PDF]

				H. D. Hemmati, I. Nakano, J. A. Lazareff, M. Masterman-Smith, D. H. Geschwind, M. Bronner-Fraser, and H. I. Kornblum Cancerous stem cells can arise from pediatric brain tumors PNAS, December 9, 2003; 100(25): 15178 - 15183. [Abstract] [Full Text] [PDF]