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

2016; Elsevier BV; Volume: 380; Issue: 1 Linguagem: Inglês

10.1016/j.canlet.2016.06.012

1872-7980

Guillem Pascual‐Pasto, Nagore G. Olaciregui, Mònica Vilà-Ubach, Sonia Paco, Carles Monterrubio, Eva Rodríguez, Ursula Winter, Mireia Batalla-Vilacis, Jaume Catalá, Héctor Salvador, Andreu Parareda, Paula Schaiquevich, Mariona Suñol, Jaume Mora, Cinzia Lavarino, Carmen de Torres, Guillermo Chantada, Ángel M. Carcaboso,

Glioma Diagnosis and Treatment

Translational research in retinoblastoma – a pediatric tumor that originates during the development of the retina – would be improved by the creation of new patient-derived models. Using tumor samples from enucleated eyes we established a new battery of preclinical models that grow in vitro in serum-free medium and in vivo in immunodeficient mice. To examine whether the new xenografts recapitulate human disease and disseminate from the retina to the central nervous system, we evaluated their histology and the presence of molecular markers of dissemination that are used in the clinical setting to detect extraocular metastases. We evaluated GD2 synthase and CRX as such markers and generated a Taqman real-time quantitative PCR method to measure CRX mRNA for rapid, sensitive and specific quantification of local and metastatic tumor burden. This approach was able to detect 1 human retinoblastoma cell in 100.000 mouse brain cells. Our research adds novel preclinical tools for the discovery of new retinoblastoma treatments for clinical translation.