Primitive neuroectodermal tumor

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It has been suggested that this article or section be merged into Ewing's sarcoma. (Discuss)

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Primitive neuroectodermal tumor Classification and external resources
ICD-O:	M9473/3
DiseasesDB	31470
eMedicine	ped/2589  neuro/326
MeSH	D018242

Primitive neuroectodermal tumor (PNET) is a rare tumor, usually occurring in children under 10 years old. It is classified into two types, based on location in the body: peripheral PNET and CNS PNET.

Contents 1 Peripheral PNET 2 PNET of the CNS 3 Ependymoblastoma 4 See also 5 References

[edit] Peripheral PNET

The peripheral PNET (pPNET) is now thought to be virtually identical to Ewing sarcoma:

"Current evidence indicates that both Ewing's sarcoma and PNET have a similar neural phenotype and, because they share an identical chromosome translocation, they should be viewed as the same tumor, differing only in their degree of neural differentiation. Tumors that demonstrate neural differentiation by light microscopy, immunohistochemistry, or electron microscopy have been traditionally labeled PNETs, and those that are undifferentiated by these analyses have been diagnosed as Ewing's sarcoma." ^[1]

[edit] PNET of the CNS

PNET of the CNS are grossly divided into supratentorial PNET and infratentorial PNET, the latter being more common. ^[2]

An example of infratentorial PNET includes medulloblastoma, which occurs in the cerebellum.

An example of supratentorial PNET includes pinealoblastoma, which occurs in the pineal region.

[edit] Ependymoblastoma

Ependymoblastoma is a synonym for PNET^[3]

[edit] See also

• Ewing family of tumors
• Ependymoma

[edit] References

1. ^ Robbins Pathologic Basis of Disease, 7th edition
2. ^ Subrata Ghosh, MD, MBBS, MS, Primitive Neuroectodermal Tumors of the Central Nervous System, eMedicine 2007.
3. ^ Ependymoblastoma at US National Institutes of Health Office of Rare Diseases

• DeVita V, Hellman S, Rosenberg S. Cancer: Principles and Practice of Oncology, 5th ed. Lippincott Williams and Wilkins (1997). p2059. ISBN 978-0397515738

v • d • e Nervous tissue tumors (ICD-O 9350-9589) Miscellaneous tumors (9350-9379) Craniopharyngioma - Pinealoma Glioma (9380-9489) Gliomatosis cerebri - Oligoastrocytoma - Choroid plexus papilloma - Ependymoma - Astrocytoma (Pilocytic astrocytoma, Glioblastoma multiforme) - Dysembryoplastic neuroepithelial tumour - Oligodendroglioma - Medulloblastoma - Primitive neuroectodermal tumor Neuroepitheliomatous (9490-9529) Ganglioneuroma - Neuroblastoma - Atypical teratoid rhabdoid tumor - Retinoblastoma - Esthesioneuroblastoma Meningiomas (9530-9539) Meningioma Nerve sheath tumor (9540-9579) Neurofibroma (Neurofibrosarcoma, Neurofibromatosis) - Schwannoma - Neurinoma - Acoustic neuroma - Neuroma see also brain tumors (though not all brain tumors are of nervous tissue)