scholarly journals PTEN and NF1 Inactivation in Schwann Cells Produces a Severe Phenotype in the Peripheral Nervous System That Promotes the Development and Malignant Progression of Peripheral Nerve Sheath Tumors

2012 ◽  
Vol 72 (13) ◽  
pp. 3405-3413 ◽  
Author(s):  
Vincent W. Keng ◽  
Eric P. Rahrmann ◽  
Adrienne L. Watson ◽  
Barbara R. Tschida ◽  
Christopher L. Moertel ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Nerve ◽  
Peripheral Nervous System ◽  
Schwann Cells ◽  
Malignant Progression ◽  
Severe Phenotype ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Prostaglandin E2 metabolism is activated in Schwann cell lines derived from human NF1 malignant peripheral nerve sheath tumors

2004 ◽  
Vol 1 (2) ◽  
pp. 149-155 ◽  
Author(s):  
GAIL D. DEADWYLER ◽  
IAN DANG ◽  
JULIE NELSON ◽  
MAYA SRIKANTH ◽  
GEORGE H. DE VRIES
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Schwann Cells ◽  
Cell Lines ◽  
Genetic Disorder ◽  
Prostaglandin E ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Cox 2

Malignant peripheral nerve sheath tumors (MPNSTs) are characteristic of Neurofibromatosis type 1 (NF1), a human genetic disorder affecting approximately 1 in 3000 individuals. The absence of neurofibromin in Schwann cells results in hyperactivation of Ras, which contributes to Schwann cell hyperplasia. However, additional intracellular abnormalities in Schwann cells might contribute to the malignancy. We now report that cell lines derived from MPNSTs secrete elevated levels of prostaglandin E2 (PGE2), express higher levels of phosphorylated mitogen-activated protein kinase (MAPK), phosphorylated cytosolic phospholipase A2 (cPLA2) and cyclooxygenase 2 (COX-2) when compared to normal adult human Schwann cells (nhSCs). PCR analysis reveals that NF1 MPNST cell lines express mRNA for both EP2 and EP4 prostaglandin E2 receptors, whereas nhSCs express only the EP4 receptor. COX-2 inhibitors and PGE2 receptor antagonists decrease the proliferation of MPNST cell lines. These results indicate that prostaglandin metabolism is activated in MPNSTs and might contribute to tumor growth in NF1.

scholarly journals Conditional Inactivation ofPtenwithEGFROverexpression in Schwann Cells Models Sporadic MPNST

Sarcoma ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Vincent W. Keng ◽  
Adrienne L. Watson ◽  
Eric P. Rahrmann ◽  
Hua Li ◽  
Barbara R. Tschida ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Peripheral Nerve ◽  
Schwann Cells ◽  
Cellular Proliferation ◽  
Regulatory Element ◽  
High Grade ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

The genetic mechanisms involved in the transformation from a benign neurofibroma to a malignant sarcoma in patients with neurofibromatosis-type-1- (NF1-)associated or sporadic malignant peripheral nerve sheath tumors (MPNSTs) remain unclear. It is hypothesized that many genetic changes are involved in transformation. Recently, it has been shown that bothphosphatase and tensin homolog(PTEN) andepidermal growth factor receptor(EGFR) play important roles in the initiation of peripheral nerve sheath tumors (PNSTs). In human MPNSTs,PTENexpression is often reduced, whileEGFRexpression is often induced. We tested if these two genes cooperate in the evolution of PNSTs. Transgenic mice were generated carrying conditional floxed alleles ofPten, andEGFRwas expressed under the control of the 2′,3′-cyclic nucleotide3′phosphodiesterase(Cnp) promoter and adesert hedgehog(Dhh) regulatory element driving Cre recombinase transgenic mice (Dhh-Cre). Complete loss ofPtenandEGFRoverexpression in Schwann cells led to the development of high-grade PNSTs.In vitroexperiments using immortalized human Schwann cells demonstrated that loss ofPTENand overexpression ofEGFRcooperate to increase cellular proliferation and anchorage-independent colony formation. This mouse model can rapidly recapitulate PNST onset and progression to high-grade PNSTs, as seen in sporadic MPNST patients.

Malignant Peripheral Nerve Sheath Tumors of the 8th Cranial Nerve Arising without Prior Irradiation

2016 ◽  
Vol 77 (S 01) ◽  
Author(s):  
Matthew Carlson ◽  
Jeffrey Jacob ◽  
Elizabeth Habermann ◽  
Amy Wagie ◽  
Aditya Raghunathan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

MR imaging of benign peripheral nerve sheath tumors

1994 ◽  
Vol 35 (3) ◽  
pp. 282-286 ◽  
Author(s):  
Veli Soderlund ◽  
H. Goranson ◽  
H. C. F. Bauer
Keyword(s):  
Mr Imaging ◽  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath
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