Klinični oddelek za hematologijo in onkologijo, Pediatrična klinika Ljubljana, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija
M. Tekavčič Pompe
Očesna klinika, Univerzitetni klinični center Ljubljana, Ljubljana
Retinoblastoma is the most common malignant tumour of the eye with an incidence of 1/15-20,000. Leukocoria and strabismus are the most common clinical presentations followed by hyphema, hypopyon, etc. The RB1 gene is demonstrated in all patients with the bilateral disease and in 85 % of cases with unilateral disease. The main diagnostic procedure is ophthalmological examination with fundoscopy, followed by ultrasound of the orbit and MRI of the eye and the central nervous system. After the clinical workup and staging, patients are divided into risk groups, on the basis of which therapy decisions are made. Patients can be treated with surgery, local therapy such as cryotherapy, brachytherapy or thermotherapy and, in cases of disease spread or bilateral disease, systemic chemotherapy. With this approach, the survival rate of retinoblastoma is more than 95% in the developed world.
Key words: retinoblastoma, local therapy, chemotherapy.