Abstract

Objective To study the effect of systemic chemotherapy and periocular carboplatin injection for orbital involvement of retinoblastoma. Methods Retrospective evaluation of 17 patients who were diagnosed and treated at the Zhongshan Ophthalmic Center,Sun Yat-Sen University (Guangzhou,China) between February 2009 and January 2011 for overt orbital involvement of retinoblastoma.They were treated by combined chemotherapy consists of intravenous chemotherapy with a CEV regimen (carboplatin,etoposide and vincristine) and local chemotherapy with periocular carboplatin injection.Of 12 patients (9 with primary orbital retinoblastoma,3 with secondary orbital retinoblastoma) received combined chemotherapy immediately after the diagnosis of orbital involvement,and the other 5 patients with primary optic nerve enlargement were enucleated upfront. Results All 12 patients who had combined chemotherapy immediately presented obvious clinical response,In which the 3 patients with secondary orbital retinoblastoma presented complete clinical response.Among the 9 patients with primary orbital retinoblastoma,8 patints were enucleated immediately after combined chemotherapy,the pathologic examination showed no viable tumor or tumor was limited to eyeball in 7patients,the optic nerve section were involved in 1 patients.Another patient relapsed in optic nerve at 6 months after refusing enucleation.Then surgery was operated,and the pathologic examination showed the optic nerve section was involved.Seven patients dead of CNS metastase in the follow-up of median 12.5 months (range 7~23).The probability of event-free survival was 48.5% at 13 months.Conclusions Systemic chemotherapy combined with local carboplatin injection is effective in orbital involvement of retinoblastoma.Which maybe replace the orbital exenteration and radiotherapy. Key words: Retinoblastoma; Orbital Involvement; Systemic Chemotherapy; Local Chemotherapy

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