Long-term outcomes for hereditary retinoblastoma
Reviewed by Nana Theodorou

Earlier diagnosis and more rigorous treatment regimens have contributed to better outcomes for patients with retinoblastoma. This study looked at 24 patients with hereditary bilateral retinoblastoma treated with systemic chemotherapy during a ten year period (2001-2011). The medical notes were reviewed for demographic data, presenting clinical signs, heredity and treatment. Chemotherapy treatment consisted of four to six cycles of vincristine, etoposide and carboplatin (VEC), administered to all newly diagnosed patients with bilateral disease. The authors detail treatment regimens in detail for the interested reader and correlate the stage of the disease with the treatment received. The majority of patients (83%) responded to the treatment and all patients completed their chemotherapy. Altogether 26% (12 of 46) of the eyes received second-line therapy with a failure rate of 35% and mortality rate 0%. The mean follow-up was 60 months. The demographic data for all patients is nicely presented in a comprehensive table to include age, gender, age at diagnosis and stage of the disease. The results indicated that group A/B retinoblastomas have a distinct chemotherapy response, while group C/D/E tumours do not respond as well. The success rate was 65%; while patients have a good prognosis for life, approximately one-third of all hereditary cases received radiation therapy or underwent enucleation. 

A 10-year experience of outcome in chemotherapy-treated hereditary retinoblastoma.
Bartuma K, Pal N, Kosek S A, et al.
ACTA OPHTHALMOLOGICA
2014;92:404-11.