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Patient-derived xenografts as preclinical neuroblastoma models

Braekeveldt, Noémie LU and Bexell, Daniel LU (2017) In Cell and Tissue Research1974-01-01+01:00 p.1-11
Abstract

The prognosis for children with high-risk neuroblastoma is often poor and survivors can suffer from severe side effects. Predictive preclinical models and novel therapeutic strategies for high-risk disease are therefore a clinical imperative. However, conventional cancer cell line-derived xenografts can deviate substantially from patient tumors in terms of their molecular and phenotypic features. Patient-derived xenografts (PDXs) recapitulate many biologically and clinically relevant features of human cancers. Importantly, PDXs can closely parallel clinical features and outcome and serve as excellent models for biomarker and preclinical drug development. Here, we review progress in and applications of neuroblastoma PDX models.... (More)

The prognosis for children with high-risk neuroblastoma is often poor and survivors can suffer from severe side effects. Predictive preclinical models and novel therapeutic strategies for high-risk disease are therefore a clinical imperative. However, conventional cancer cell line-derived xenografts can deviate substantially from patient tumors in terms of their molecular and phenotypic features. Patient-derived xenografts (PDXs) recapitulate many biologically and clinically relevant features of human cancers. Importantly, PDXs can closely parallel clinical features and outcome and serve as excellent models for biomarker and preclinical drug development. Here, we review progress in and applications of neuroblastoma PDX models. Neuroblastoma orthotopic PDXs share the molecular characteristics, neuroblastoma markers, invasive properties and tumor stroma of aggressive patient tumors and retain spontaneous metastatic capacity to distant organs including bone marrow. The recent identification of genomic changes in relapsed neuroblastomas opens up opportunities to target treatment-resistant tumors in well-characterized neuroblastoma PDXs. We highlight and discuss the features and various sources of neuroblastoma PDXs, methodological considerations when establishing neuroblastoma PDXs, in vitro 3D models, current limitations of PDX models and their application to preclinical drug testing.

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author
organization
publishing date
type
Contribution to journal
publication status
in press
subject
keywords
Mouse model, Neuroblastoma, Patient-derived xenograft (PDX), Pediatric cancer
in
Cell and Tissue Research1974-01-01+01:00
pages
1 - 11
publisher
Springer
external identifiers
  • scopus:85029577622
ISSN
0302-766X
DOI
10.1007/s00441-017-2687-8
language
English
LU publication?
yes
id
958b9899-d426-49e2-8887-876dd8e03ad2
date added to LUP
2017-10-12 10:36:20
date last changed
2018-01-12 03:00:08
@article{958b9899-d426-49e2-8887-876dd8e03ad2,
  abstract     = {<p>The prognosis for children with high-risk neuroblastoma is often poor and survivors can suffer from severe side effects. Predictive preclinical models and novel therapeutic strategies for high-risk disease are therefore a clinical imperative. However, conventional cancer cell line-derived xenografts can deviate substantially from patient tumors in terms of their molecular and phenotypic features. Patient-derived xenografts (PDXs) recapitulate many biologically and clinically relevant features of human cancers. Importantly, PDXs can closely parallel clinical features and outcome and serve as excellent models for biomarker and preclinical drug development. Here, we review progress in and applications of neuroblastoma PDX models. Neuroblastoma orthotopic PDXs share the molecular characteristics, neuroblastoma markers, invasive properties and tumor stroma of aggressive patient tumors and retain spontaneous metastatic capacity to distant organs including bone marrow. The recent identification of genomic changes in relapsed neuroblastomas opens up opportunities to target treatment-resistant tumors in well-characterized neuroblastoma PDXs. We highlight and discuss the features and various sources of neuroblastoma PDXs, methodological considerations when establishing neuroblastoma PDXs, in vitro 3D models, current limitations of PDX models and their application to preclinical drug testing.</p>},
  author       = {Braekeveldt, Noémie and Bexell, Daniel},
  issn         = {0302-766X},
  keyword      = {Mouse model,Neuroblastoma,Patient-derived xenograft (PDX),Pediatric cancer},
  language     = {eng},
  month        = {09},
  pages        = {1--11},
  publisher    = {Springer},
  series       = {Cell and Tissue Research1974-01-01+01:00},
  title        = {Patient-derived xenografts as preclinical neuroblastoma models},
  url          = {http://dx.doi.org/10.1007/s00441-017-2687-8},
  year         = {2017},
}