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A 10-year experience of outcome in chemotherapy-treated hereditary retinoblastoma
Karolinska Inst, St Erik Eye Hosp, Dept Vitreoretinal Dis, Stockholm, Sweden..
Karolinska Inst, Dept Pediat, Astrid Lindgren Childrens Hosp, S-10401 Stockholm, Sweden..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences. Uppsala Univ, Uppsala, Sweden..
Karolinska Inst, Dept Pediat, Astrid Lindgren Childrens Hosp, S-10401 Stockholm, Sweden..
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2014 (English)In: Acta Ophthalmologica, ISSN 1755-375X, E-ISSN 1755-3768, Vol. 92, no 5, 404-411 p.Article in journal (Refereed) Published
Abstract [en]

Purpose: The aim is to report the 10-year retrospective experience of systemic chemotherapy for a population-based group of patients with hereditary retinoblastoma at a national referral centre. The outcomes include control rates, treatment side-effects, adjuvant therapy, failure rate, survival, secondary cancers and visual acuity. Methods: All patients (n = 24, 46 eyes) diagnosed with retinoblastoma and treated with systemic chemotherapy at a national referral centre during 2001-2011 were included. Data were extracted from medical records. Results: The patients were followed for a mean of 60 months (range 13-144). Four-six cycles of VEC was administered to all newly diagnosed group B/C/D/E eyes with bilateral disease and 83% (38 of 46) responded to the treatment. None of the patients discontinued chemotherapy because of adverse reactions. Altogether 26% (12 of 46) of the eyes received second-line therapy (other than thermotherapy, cryotherapy and chemotherapy). The failure rate was 35% (16 of 46) and mortality rate 0%. None of the patients developed CNS manifestations (metastases or trilateral retinoblastoma). One of the patients developed a second primary tumour (osteosarcoma) 4 years following retinoblastoma diagnosis. Altogether 17% (4 of 24) patients received radiation therapy, 28% (13 of 46) of the eyes had to be enucleated, and one patient underwent bilateral enucleation. The age-correlated visual acuity was mean of 73% of expected visual acuity. Conclusion: 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.

Place, publisher, year, edition, pages
2014. Vol. 92, no 5, 404-411 p.
Keyword [en]
chemotherapy, failure rate, focal therapy, hereditary retinoblastoma, visual acuity
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URN: urn:nbn:se:uu:diva-306339DOI: 10.1111/aos.12282ISI: 000339482700025PubMedID: 24119165OAI: oai:DiVA.org:uu-306339DiVA: diva2:1047261
Available from: 2016-11-17 Created: 2016-10-27 Last updated: 2016-11-17

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