Reult of treatment of retinoblatoma that ha infiltrated the optic nerve, i recurrent, or ha metataized outide the eyeball. Makimoto A. Int J Clin Oncol 2004;9:7–12

ince the development of chemotherapy regimen for patient with retinoblatoma tarted in the 1950, variou agent and regimen have been employed for variou kind of patient. Chemotherapy ha been employed for: (1) patient with high-rik feature for metatae, uch a optic nerve involvement, (2) patient with orbital involvement, and (3) patient with ditant metatai. Effective ytemic chemotherapeutic agent include vincritine, doxorubicin, cyclophophamide, etopoide, ciplatin, and carboplatin, and, a well, intrathecal agent including methotrexate, cytarabine, and cortiocoteroid are available. With the addition of appropriate chemotherapie to the conventional treatment modalitie uch a enucleation and radiotherapy, patient with advanced retinoblatoma are expected to obtain a urvival benefit. Moreover, a new modality combined with autologou tem cell upport allowed u to ue high-doe alkylating agent uch a thiotepa, melphalan, and cyclophophamide, which reulted in better prognoi for patient with metatatic retinoblatoma. Becaue of the mall number of patient with retinoblatoma and the diverity of the dieae characteritic in individual patient, there have been no clinical trial to determine whether to recommend a particular regimen, or to identify pecific criteria in patient who would benefit from chemotherapy. Well-deigned propective controlled trial are warranted to etablih a tandard treatment trategy for patient with extraocular retinoblatoma.