INFANTILE RETINOBLASTOMA MORBIDITY IN PERM KRAI

Aim. To analyze the cases of retinoblastoma in Perm Krai. Materials and methods. Retrospective analysis of case histories of 29 children diagnosed retinoblastoma who had undergone medical examination at Perm Children's Oncohematological Center during 18 years (1996-2013) was performed. Results. Out of 29 children aged 1 year 5 months to 18 years who were diagnosed retinoblastoma on 37 eyes there were 15 (51,7 %) boys and 14 (48,3 %) girls. Nearly in half of cases retinoblastoma was diagnosed at the first year of life - 48,3 %. More often the left eyes were affected - 24 (65 %); 15 children live in the towns of Perm Krai (51,7 %) and half of them live in Perm. Burdened heredity was detected only in 1 (3,4 %) child with monocular form, 1 case of retinoblastoma - in 2 stepbrothers (by father) with binocular retinoblastoma. Occupational hazard of parents was noted in 4 families; concomitant diseases were found in 15 mothers (51,7 %). Monocular form of retinoblastoma was registered in 21 children, binocular - in 8 children; 26 (70,3 %) injured eyes were enucleated in 25 children with the following polychemotherapy; organ-preserving methods of treatment (polychemotherapy and radial therapy) were applied on 7 eyes. Parents of 2 children with binocular form of retinoblastoma refused from therapy, and as a result of severe state of health against the background of generalized metastatic disease, these children died. Conclusions. Modern diagnostic equipment which appeared at the regional center and oncological suspicion among pediatric ophthalmologists give hope of earlier diagnosis and organ-preserving treatment. It is necessary to carry out medical-genetic consultation among parents of risk group.

Retinoblastoma, organ-preserving treatment, enucleation