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A 10-year experienc...
A 10-year experience of outcome in chemotherapy-treated hereditary retinoblastoma
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- Bartuma, Katarina (author)
- Karolinska Inst, St Erik Eye Hosp, Dept Vitreoretinal Dis, Stockholm, Sweden.
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- Pal, Niklas (author)
- Karolinska Institutet
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- Kosek, Sonja (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Uppsala Univ, Uppsala, Sweden.
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- Holm, Stefan (author)
- Karolinska Institutet
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- All-Ericsson, Charlotta (author)
- Karolinska Inst, St Erik Eye Hosp, Dept Vitreoretinal Dis, Stockholm, Sweden.
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Karolinska Institutet Karolinska Inst, St Erik Eye Hosp, Dept Vitreoretinal Dis, Stockholm, Sweden (creator_code:org_t)
- 2013-10-07
- 2014
- English.
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In: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 92:5, s. 404-411
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Abstract
Subject headings
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Oftalmologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Ophthalmology (hsv//eng)
Keyword
- chemotherapy
- failure rate
- focal therapy
- hereditary retinoblastoma
- visual acuity
Publication and Content Type
- ref (subject category)
- art (subject category)
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