Engraftment and growth of patient-derived retinoblastoma tumour in severe combined immunodeficiency mice

The development of an in vivo model or retinoblastoma could ve important fo r studying its biological behaviour and developing novel therapeutic strate gies. We examined the ability of patient-derived retinoblastoma cells to gr ow and disseminate in severe combined immunodeficiency CB-17-SCID mice afte r subcutaneous (s.c.) inoculation without conditioning treatment. 24/30 (80 %) of patient-derived rumours engrafted and grew as s.c. nodules in SCID mi ce. Whilst most xenografted rumours appeared to be localised, by PCR assay a positive DNA band of human minisatellite region (YNZ.22) was determined i n the bone marrow of 19/25 (76%), in the spleen of 14/25 (56%) and in the l iver of 16/25 (64%) mice, respectively, indicating dissemination to distant organs. Cytogenetic analysis demonstrated i(6p) in 5/12 (42%) and trisomy 1 or 1q abnormalities in 8/12 (67%) of the xenografted tumour samples studi ed, respectively, suggesting that retinoblastoma tumour cells maintain thei r cytogenetic abnormalities following adoptive growth in SCID mice. In this report we demonstrate the ability to propagate human primary retinoblastom a cells in SCID mice after s.c. inoculation and suggest the possibility of using the SCID mouse model to study the intrinsic biological behaviour of h uman retinoblastoma and to develop novel therapeutic strategies in the trea tment of this disease. (C) 2000 Published by Elsevier Science Ltd. All righ ts reserved.