Primitive neuroectodermal tumor
| Primitive neuroectodermal tumor | |
|---|---|
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| Micrograph of an H&E stained section of a peripheral PNET. |
Primitive neuroectodermal tumor (PNET) is a malignant (cancerous) neural crest tumor.[1] It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%.[2]
It gets its name because the majority of the cells in the tumor are derived from neuroectoderm, but have not developed and differentiated in the way a normal neuron would, and so the cells appear "primitive".
PNET belongs to the Ewing family of tumors.
Genetics
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Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis.[3] The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 Large T- antigen, other than p53.[4]
Diagnosis
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Treatment
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See also
References
- ^ "primitive neuroectodermal tumor" at Dorland's Medical Dictionary
- ^ Smoll, N. R. (2012). "Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs)". Cancer. 118 (5): 1313–1322. doi:10.1002/cncr.26387. PMID 21837678.
- ^ Eibl RH, Kleihues P, Jat PS, Wiestler OD (March 1994). "A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen". Am. J. Pathol. 144 (3): 556–64. PMC 1887088. PMID 8129041.
- ^ Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P (November 1991). "p53 mutations in nonastrocytic human brain tumors". Cancer Res. 51 (22): 6202–5. PMID 1933879.