Retinoblastoma: a patient’s story

Relevance. Development and application of novel treatment methods for patients with retinoblastoma (Rb) is a great task since it may provide higher rates of eye salvage and reduce adverse effects of current therapy.

Aim of the study was to represent the history of Rb eye-sparing treatment in Russia and worldwide through the one clinical case.

Materials and methods. A child was treated by systemic chemotherapy, plaque brachytherapy using non-described techniques, cryotherapy and external beam radiotherapy. Intravitreal chemotherapy was approached for treatment of vitreous seeding. Vitreoretinal surgery with melphalan irrigation was successfully performed after failure of vitreous hemorrhage conservative treatment and parents’ refusal to indicated enucleation. The child died from CNS disseminated esthesioneuroblastoma.

Results. The use of various treatment modalities helped to save the single eye.

Conclusions. Intravitreal chemotherapy was introduced in Russia for the first time. Performed vitreoretinal surgery with melphalan irrigation was proved to be a safe and effective technique in case of vitreous hemorrhage in the eye with Rb. External beam therapy is associated with fatal second tumors development.

1. Dimaras H., Corson T.W., Cobrinik D., White A., Zhao J., Munier F.L., Abramson D.H., Shields C.L., Chantada G.L., Njuguna F., Gallie B.L. Retinoblastoma. Nat Rev Dis Prim 2015;1(1):15021. doi: 10.1038/nrdp.2015.21.

2. Ushakova T.L., Тrofi mov I.A., Gorovtsova O.V., Yarovoy A.A., Saakyan S.V., Letyagin I.A., Маtinyan N.V., Кukushkin A.V., Маrtynov L.A., Pogrebnyakov I.V., Ivanova O.A., Serov Y.A., Yarovaya V.A., Glekov I.V., Virshke E.R., Dolgushin B.I., Polyakov V.G. A New era of organ-preserving treatment in pediatric intraocular retinoblastoma in Russia: a multicenter cohort study. Oncopediatrics 2018;5(1):51–69. doi: 10.15690/onco.v5i1.1866.

3. Berry J.L., Kogachi K., Jubran R., Kim J.W. Loss of fundus view as an indication for secondary enucleation in retinoblastoma. Pediatr Blood Cancer 2018;65(4):e26908. doi: 10.1002/pbc.26908.

4. Yu C.L., Tucker M.A., Abramson D.H., Furukawa K., Seddon J. M., Stovall M., Fraumeni J. F., Kleinerman R.A. Cause-specifi c mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst 2009;101(8):581–91. doi: 10.1093/jnci/djp046.

5. Kim J.-Y., Park Y. Treatment of retinoblastoma: the role of external beam radiotherapy. Yonsei Med J 2015;56(6):1478. doi: 10.3349/ymj.2015.56.6.1478.

6. Demirci H. Histopathologic fi ndings in eyes with retinoblastoma treated only with chemoreduction. Arch Ophthalmol 2003;121(8):1125. doi: 10.1001/archopht.121.8.1125.

7. Kaneko A. Eye-preservation treatment of retinoblastoma with vitreous seeding. Jpn J Clin Oncol 2003;33(12):601–7. doi: 10.1093/jjco/hyg113.

8. Honavar S.G. Intraocular surgery after treatment of retinoblastoma. Arch Ophthalmol 2001;119(11):1613. doi: 10.1001/archopht.119.11.1613.

9. Ohshima K.-I., Kaneko T., Takagi S., Kaneko A., Yokouchi Y., Takeuchi S. Clinicopathological investigation of a retinoblastoma eye enucleated after vitreous surgery with melphalan perfusion. Jpn J Ophthalmol 2009;53(2):186–8. doi: 10.1007/s10384-008-0636-5.

10. Shimoda Y., Hamano R., Ishihara K., Shimoda N., Hagimura N., Akiyama H., Kishi S., Kaneko A. Eff ects of intraocular irrigation with melphalan on rabbit retinas during vitrectomy. Graefes Arch Clin Exp Ophthalmol 2008;246(4):501–8. doi: 10.1007/s00417-007-0685-3.

11. Yarovoy A.A., Ushakova T.L., Gorshkov I.M., Polyakov V.G., Golubeva O.V., Gorovtsova O.V., Krivovyaz O.S. Intraocular surgery with melphalan irrigation for vitreous hemorrhage in an only eye with retinoblastoma. Eur J Ophthalmol 2016;26(1):17–9. doi: 10.5301/ejo.5000683.

12. Solenova L.G. Second primary malignancies in cancer survivors: epidemiology, role of anticancer therapy. Adv Mol Oncol 2016;3(3):30–43. doi: 10.17650/2313-805X-2016-3-3-30-43.

13. Rubenfeld M., Abramson D.H., Ellsworth R.M., Kitchin F.D. Unilateral vs. bilateral retinoblastoma: correlations between age at diagnosis and stage of ocular disease. Ophthalmology 1986;93(8):1016–9. doi: 10.1016/s0161-6420(86)33629-7.

14. Dulguerov P., Allal A.S., Calcaterra T.C. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001;2(11):683–90. doi: 10.1016/S1470-2045(01)00558-7.

15. Carey R.M., Godovchik J., Workman A.D., Kuan E.C., Parasher A.K., Chen J., Palmer J.N., Adappa N.D., Newman, J.G., Brant J.A. Patient, disease, and treatment factors associated with overall survival in esthesioneuroblastoma. Int Forum Allergy Rhinol 2017;7(12):1186–94. doi: 10.1002/alr.22027.

16. Morita A., Ebersold M.J., Olsen K.D., Foote R.L., Lewis J.E., Quast L.M. Esthesioneuroblastoma: prognosis and management. Neurosurgery 1993;32(5):706–15. doi: 10.1227/00006123-199305000-00002.