Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Großschaedl, C.
Management and outcome analysis of patients with Retinoblastoma treated according to the prospective
RB A-2003 and RB-Registry studies at a single center
Humanmedizin; [ Diplomarbeit ] Medizinische Universitaet Graz; 2022. pp. 90
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- Autor*innen der Med Uni Graz:
- Betreuer*innen:
- Seidel Markus
- Steltner Birgit
- Altmetrics:
- Abstract:
- Introduction With an incidence of 16.000-18.000 newly diagnosed children per year, retinoblastoma is the most common intraocular tumor in childhood. The Department of Ophthalmology in close cooperation with the Division of Paediatric Haematology/ Oncology at the Medical University of Graz are treating children with retinoblastoma since the 19070s. From 2003 onwards, all children diagnosed with intraocular retinoblastoma were enrolled in the therapy-optimisation study RB A-2003; since 2014 patients with any stage of disease who are residents of Austria are included in the RB-Registry of the University of Essen. The aim of this diploma thesis is to evaluate patient characteristics, outcome and therapy management within these two studies. Patients and Methods A total of 59 patients (85 eyes) treated at a single-centre between 2003 and 2020 were retrospectively analysed. The cohort included 32 females and 27 males, who were first diagnosed with retinoblastoma between the ages of 0-10. The median follow-up time was 3.96 years (min-max: 0.19-13.66). The relevant data were gathered from medical records and retrieved by retrospective chart review in accordance with current guidelines for good clinical practice. To assess the efficacy of the management, detailed numbers of overall survival, eye-survival and recurrences were analysed. A special focus was placed on local chemotherapeutic options. Data were evaluated in relation to treatment measures, expressed by Kaplan-Meier curves, comparing, e.g. hereditary versus non-hereditary cases, or RB A-2003 versus RB-Registry. Detailed patient characteristics are presented with descriptive statistics. Results All in all, 59 patients with retinoblastoma were diagnosed in Graz during the time frame of the study. Of these, 27 were enrolled in RB A-2003 and 32 in RB-Registry. A total of 33 patients were affected with unilateral eye involvement, 26 with bilateral. During the observational period, the overall survival was 100%. 36 of 85 eyes were enucleated (RB A-2003: n=16, RB-Registry: n=20), 23 of them primary and 13 secondary. Twelve patients received adjuvant chemotherapy after the enucleation. Due to a higher stage of disease, 2 patients were treated with radiotherapy and autologous stem cell transplantation. Local chemotherapy was administered on 12 eyes. A recurrence, new onset or insufficient regression was observed in a total of 30 eyes (RB A-2003: n=18, RB-Registry: n=12). Conclusion and Discussion These data represent a comprehensive, retrospective analysis of children with retinoblastoma treated at a large tertiary center in Austria. Even though the overall survival rate is 100%, the number of enucleated eyes is still high, compared to international data. This might be explained by the infrequent use of treatment modalities including local chemotherapy in Graz. Therefore, the development of local chemotherapeutic options could be indicated.