Preclinical platform of retinoblastoma xenografts recapitulating human disease and molecular markers of dissemination

Clinical, Genomic, and Pharmacological Study of MYCN-Amplified RB1 Wild-Type Metastatic Retinoblastoma

“…Additionally, the model recapitulated the clinical behavior of Patient 2, as almost all of HPG-RBG1 xenografts showed lymph node infiltration without brain involvement. In contrast, Y79 CDXs showed a significant higher frequency of optic nerve and brain infiltration, as expected from previous reports and based on the proximity of the anatomical structures, but the lymph nodes were free of tumor [26,29]. Lymph node dissemination was previously reported in two transgenic mice models with RB1 −/− retinoblastoma and MYCN amplification [30].…”

Clinical, Genomic, and Pharmacological Study of MYCN-Amplified RB1 Wild-Type Metastatic Retinoblastoma

“…Additionally, the model recapitulated the clinical behavior of Patient 2, as almost all of HPG-RBG1 xenografts showed lymph node infiltration without brain involvement. In contrast, Y79 CDXs showed a significant higher frequency of optic nerve and brain infiltration, as expected from previous reports and based on the proximity of the anatomical structures, but the lymph nodes were free of tumor [26,29]. Lymph node dissemination was previously reported in two transgenic mice models with RB1 −/− retinoblastoma and MYCN amplification [30].…”

Injectable hydrogel-mediated co-delivery of oncolytic adenovirus and melphalan for retinoblastoma control and vision preservation

“…The cone-rod homeobox (CRX) transcription factor, a marker for cone lineage cells, is expressed in both RB and normal retina but not in other tissues. Thus, CRX mRNA expression has been used as a marker for brain metastasis in RB models 43 , 44 , 45 , 46 . We analyzed brain samples obtained from tumor-bearing mice either after enucleation or on Day 100.…”

Tridimensional Retinoblastoma Cultures as Vitreous Seeds Models for Live-Cell Imaging of Chemotherapy Penetration

“…Then, we first confirmed the origin of the cell lines by different genomic analysis and the identification of the expression of markers of retinal and neuronal tumor origin to sustain the basis of all further developments. Importantly, cell-growth conditions avoiding the use of serum for culturing the cells from the biopsy have been shown to dramatically improve tumor cell selection and to avoid differentiation of the cell culture to a different phenotype than the parental tumors [18,19]. Moreover, it is important to mention that cell-growth conditions and the cell line passage number may result in different pharmacological sensitivity of the cell cultures as previously reported for retinoblastoma and other tumors [27,28].…”

Clinical, Genomic, and Pharmacological Study of MYCN-Amplified RB1 Wild-Type Metastatic Retinoblastoma

“…Additionally, the model recapitulated the clinical behavior of Patient 2, as almost all of HPG-RBG1 xenografts showed lymph node infiltration without brain involvement. In contrast, Y79 CDXs showed a significant higher frequency of optic nerve and brain infiltration, as expected from previous reports and based on the proximity of the anatomical structures, but the lymph nodes were free of tumor [26,29]. Lymph node dissemination was previously reported in two transgenic mice models with RB1 −/− retinoblastoma and MYCN amplification [30].…”

Injectable hydrogel-mediated co-delivery of oncolytic adenovirus and melphalan for retinoblastoma control and vision preservation

“…The cone-rod homeobox (CRX) transcription factor, a marker for cone lineage cells, is expressed in both RB and normal retina but not in other tissues. Thus, CRX mRNA expression has been used as a marker for brain metastasis in RB models 43 , 44 , 45 , 46 . We analyzed brain samples obtained from tumor-bearing mice either after enucleation or on Day 100.…”

Tridimensional Retinoblastoma Cultures as Vitreous Seeds Models for Live-Cell Imaging of Chemotherapy Penetration

“…Then, we first confirmed the origin of the cell lines by different genomic analysis and the identification of the expression of markers of retinal and neuronal tumor origin to sustain the basis of all further developments. Importantly, cell-growth conditions avoiding the use of serum for culturing the cells from the biopsy have been shown to dramatically improve tumor cell selection and to avoid differentiation of the cell culture to a different phenotype than the parental tumors [18,19]. Moreover, it is important to mention that cell-growth conditions and the cell line passage number may result in different pharmacological sensitivity of the cell cultures as previously reported for retinoblastoma and other tumors [27,28].…”

Clinical, Genomic, and Pharmacological Study of MYCN-Amplified RB1 Wild-Type Metastatic Retinoblastoma

“…Additionally, the model recapitulated the clinical behavior of Patient 2, as almost all of HPG-RBG1 xenografts showed lymph node infiltration without brain involvement. In contrast, Y79 CDXs showed a significant higher frequency of optic nerve and brain infiltration, as expected from previous reports and based on the proximity of the anatomical structures, but the lymph nodes were free of tumor [26,29]. Lymph node dissemination was previously reported in two transgenic mice models with RB1 −/− retinoblastoma and MYCN amplification [30].…”

Injectable hydrogel-mediated co-delivery of oncolytic adenovirus and melphalan for retinoblastoma control and vision preservation

“…The cone-rod homeobox (CRX) transcription factor, a marker for cone lineage cells, is expressed in both RB and normal retina but not in other tissues. Thus, CRX mRNA expression has been used as a marker for brain metastasis in RB models 43 , 44 , 45 , 46 . We analyzed brain samples obtained from tumor-bearing mice either after enucleation or on Day 100.…”

Tridimensional Retinoblastoma Cultures as Vitreous Seeds Models for Live-Cell Imaging of Chemotherapy Penetration

“…Then, we first confirmed the origin of the cell lines by different genomic analysis and the identification of the expression of markers of retinal and neuronal tumor origin to sustain the basis of all further developments. Importantly, cell-growth conditions avoiding the use of serum for culturing the cells from the biopsy have been shown to dramatically improve tumor cell selection and to avoid differentiation of the cell culture to a different phenotype than the parental tumors [18,19]. Moreover, it is important to mention that cell-growth conditions and the cell line passage number may result in different pharmacological sensitivity of the cell cultures as previously reported for retinoblastoma and other tumors [27,28].…”