BACKGROUND: Orbital involvement is a rare occurrence in retinoblastoma but still carries a bad prognosis. The aim of this study was to define more clearly the role of chemotherapy in this advanced disease. METHODS: Between 1977 and 1991, 33 patients were treated at the Services de Pédiatrie at Institut Curie (Paris, France) for orbital involvement of retinoblastoma, which was isolated in 20 patients and associated with metastases in 13 patients (outside the central nervous system [CNS], 6; within the CNS, 7). Treatment included chemotherapy for 33 patients, irradiation of the orbit for 23 patients, and intrathecal chemotherapy and/or CNS irradiation in selected patients. RESULTS: The plateau phase of the survival curve was reached at 15 months, with a survival of 34% plus or minus 8%. The disease free interval was longer when patients had no CNS disease (P < 0.05). Twenty of the 21 recurrences (95%) occurred within 1 year after diagnosis of orbital involvement. CONCLUSIONS: Intensive chemotherapy using cyclophosphamide, platinum compounds, epipodophyllotoxins, doxorubicin, and vincristine was effective in orbital involvement of retinoblastoma even with associated extra-CNS metastases. However, associated CNS disease still carries a bad prognosis, and long term follow-up is necessary to evaluate the risk of a second tumor.
BACKGROUND: Orbital involvement is a rare occurrence in retinoblastoma but still carries a bad prognosis. The aim of this study was to define more clearly the role of chemotherapy in this advanced disease. METHODS: Between 1977 and 1991, 33 patients were treated at the Services de Pédiatrie at Institut Curie (Paris, France) for orbital involvement of retinoblastoma, which was isolated in 20 patients and associated with metastases in 13 patients (outside the central nervous system [CNS], 6; within the CNS, 7). Treatment included chemotherapy for 33 patients, irradiation of the orbit for 23 patients, and intrathecal chemotherapy and/or CNS irradiation in selected patients. RESULTS: The plateau phase of the survival curve was reached at 15 months, with a survival of 34% plus or minus 8%. The disease free interval was longer when patients had no CNS disease (P < 0.05). Twenty of the 21 recurrences (95%) occurred within 1 year after diagnosis of orbital involvement. CONCLUSIONS: Intensive chemotherapy using cyclophosphamide, platinum compounds, epipodophyllotoxins, doxorubicin, and vincristine was effective in orbital involvement of retinoblastoma even with associated extra-CNS metastases. However, associated CNS disease still carries a bad prognosis, and long term follow-up is necessary to evaluate the risk of a second tumor.
Authors: Carlos A Leal-Leal; Roberto Rivera-Luna; Martha Flores-Rojo; Juan C Juárez-Echenique; Juan C Ordaz; Jorge Amador-Zarco Journal: Clin Transl Oncol Date: 2006-01 Impact factor: 3.405
Authors: Nicolas Alessandro Yannuzzi; Jasmine H Francis; Brian P Marr; Irina Belinsky; Ira J Dunkel; Yves Pierre Gobin; David Harold Abramson Journal: JAMA Ophthalmol Date: 2015-09 Impact factor: 7.389
Authors: Hervé J Brisse; Pim de Graaf; Paolo Galluzzi; Kristel Cosker; Philippe Maeder; Sophia Göricke; Firazia Rodjan; Marcus C de Jong; Alexia Savignoni; Isabelle Aerts; Laurence Desjardins; Annette C Moll; Theodora Hadjistilianou; Paolo Toti; Paul van der Valk; Jonas A Castelijns; Xavier Sastre-Garau Journal: Eur Radiol Date: 2014-11-30 Impact factor: 5.315
Authors: C Levy; F Doz; E Quintana; H Pacquement; J Michon; P Schlienger; P Validire; B Asselain; L Desjardins; J M Zucker Journal: Br J Ophthalmol Date: 1998-10 Impact factor: 4.638