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Cellular Classification
Current Clinical Trials
The classification of brain tumors is based on both histopathologic
characteristics and location in the brain. Undifferentiated neuroectodermal
tumors of the cerebellum have historically been referred to as
medulloblastomas, while tumors of identical histology in the pineal region are
diagnosed as pineoblastomas, and cortical lesions have been called central neuroblastomas or cortical primitive neuroectodermal tumors. Studies have suggested that the tumor cells of medulloblastomas and
supratentorial primitive neuroectodermal tumors have different molecular genetic aberrations.[1,2] This group also includes
some tumors formerly called central neuroblastomas. The nomenclature of
pediatric brain tumors is controversial and potentially confusing. Some
pathologists advocate abandoning the traditional morphologically based
classifications such as medulloblastoma in favor of a terminology that relies
more extensively on the phenotypic characteristics of the tumor. In such a
system, medulloblastoma is referred to as primitive neuroectodermal tumor and
then subdivided on the basis of cellular differentiation. The World Health Organization 2000 classification of brain tumors maintains the term
medulloblastoma for posterior fossa undifferentiated tumors.[3] It also
maintains separate categories for cerebral primitive neuroectodermal tumors and
for pineal small round cell tumors (pineoblastomas). The pathologic
classification of pediatric brain tumors is a specialized area that is
evolving; review of the diagnostic tissue by a neuropathologist who
has particular expertise in this area is strongly recommended.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References
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Russo C, Pellarin M, Tingby O, et al.: Comparative genomic hybridization in patients with supratentorial and infratentorial primitive neuroectodermal tumors. Cancer 86 (2): 331-9, 1999.
[PUBMED Abstract]
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Nicholson JC, Ross FM, Kohler JA, et al.: Comparative genomic hybridization and histological variation in primitive neuroectodermal tumours. Br J Cancer 80 (9): 1322-31, 1999.
[PUBMED Abstract]
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Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.
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