WordNet

malignant tumor of the neuroepithelium

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/08/05 21:56:48」(JST)

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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.

Classification

It is classified into two types, based on location in the body: peripheral PNET and CNS PNET.

Peripheral PNET

CD99 staining of tissue from 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."^[3]

PNET of the CNS

Supratentorial central PNET in a 5-year-old patient

PNET of the CNS generally refer to supratentorial PNETs.

In the past medulloblastomas were considered PNETs; however, they are genetically, transcriptionally and clinically distinct. As such, "infratentorial" PNETs are now referred to as medulloblastoma^{[citation needed]}.

Pineoblastomas are embryonal tumours originating in the pineal gland and are likely distinct from supratentorial PNETs.

Model

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.^[4] 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.^[5]

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. edit
^ Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) Robbins & Cotran Pathologic Basis of Disease (7th ed.). Saunders. Page 1301. ISBN 0-7216-0187-1.
^ 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.

English Journal

Radiologic and clinicopathologic findings of peripheral primitive neuroectodermal tumors.

Ba L1, Tan H2, Xiao H3, Guan Y4, Gao J3, Gao X5.
Acta radiologica (Stockholm, Sweden : 1987).Acta Radiol.2015 Jul;56(7):820-8. doi: 10.1177/0284185114539321. Epub 2014 Jul 29.
BACKGROUND: Primitive neuroectodermal tumors (PNETs) constitute a rare type of malignant neuroectodermal tumors that have chromosomal translocations identical to Ewing's sarcoma (ES), and the characteristics of this disease remain unclear.PURPOSE: To describe the clinical, radiological, and patholog
PMID 25073463

Sox10-a marker for not only schwannian and melanocytic neoplasms but also myoepithelial cell tumors of soft tissue: a systematic analysis of 5134 tumors.

Miettinen M1, McCue PA, Sarlomo-Rikala M, Biernat W, Czapiewski P, Kopczynski J, Thompson LD, Lasota J, Wang Z, Fetsch JF.
The American journal of surgical pathology.Am J Surg Pathol.2015 Jun;39(6):826-35. doi: 10.1097/PAS.0000000000000398.
Sox10 transcription factor is expressed in schwannian and melanocytic lineages and is important in their development and can be used as a marker for corresponding tumors. In addition, it has been reported in subsets of myoepithelial/basal cell epithelial neoplasms, but its expression remains incompl
PMID 25724000

TLR3 triggering regulates PD-L1 (CD274) expression in human neuroblastoma cells.

Boes M1, Meyer-Wentrup F2.
Cancer letters.Cancer Lett.2015 May 28;361(1):49-56. doi: 10.1016/j.canlet.2015.02.027. Epub 2015 Feb 16.
Neuroblastoma is the most common extracranial solid tumor in children, causing 12% of all pediatric cancer mortality. Neuroblastoma specific T-cells have been detected in patients, but usually fail to attack and eradicate the tumors. Tumor immune evasion may thus play an important role in neuroblast
PMID 25697485

Japanese Journal

十二指腸より発生したEwing肉腫ファミリー腫瘍の1例

日本臨床外科学会雑誌 76(4), 743-748, 2015
NAID 130005106544

末梢神経未分化神経外胚葉性腫瘍と鑑別を要した原発性皮膚未分化大細胞リンパ腫の1例

Skin Cancer 28(3), 259-263, 2014
NAID 130004697512

MIC-2およびEWS遺伝子転座が診断根拠となった原発性頭蓋内Ewing肉腫/末梢性primitive neuroectodermal tumorの1例

脳神経外科ジャーナル 20(10), 761-767, 2011-10-20
NAID 110008750603

Related Pictures

★リンクテーブル★

リンク元	「peripheral primitive neuroectodermal tumor」「neuroepithelioma」「peripheral neuroectodermal tumor」

「peripheral primitive neuroectodermal tumor」

Primitive neuroectodermal tumor
	Micrograph of an H&E stained section of a peripheral PNET.
Classification and external resources
ICD-O	M9473/3
DiseasesDB	31470
eMedicine	ped/2589 neuro/326
MeSH	D018242