Diffuse infiltrating retinoblastoma: a multicentre,international, data-sharing studyстатья
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Дата последнего поиска статьи во внешних источниках: 23 января 2026 г.
Аннотация:ABSTRACTBackground To determine the unique clinicalcharacteristics and treatment outcomes of diffuseinfiltrating retinoblastoma (DIR).Methods This international, multicentre, registry-basedretrospective case series analysed pooled datafrom January 2001 to December 2013, including132 eyes from 132 patients with DIR.Results Among 2854 eyes with retinoblastoma,132 (4.6%; 95% CI, 3.9 to 5.5) had DIR. Themedian age at diagnosis for DIR patients was24 months (IQR, 15–33), with no bilateralcases of DIR. The American Joint Committee onCancer staging showed 4.5% cT2 and 95.5%cT3 categories, with no cT1 or cT4 cases. Clinicalfeatures associated with DIR included secondaryglaucoma (67%), retinal detachment (38%),diffuse vitreous seeds (37%), anterior segmentinvolvement (24%), vitreous haemorrhage (50%)and hyphema (6%). Primary enucleation was thepredominant treatment (81%), while 19% initiallyreceived systemic chemotherapy, with 6% requiringsubsequent enucleation. The 5-yearKaplan-Meiersurvival rate for cT3 DIR was 82% (95% CI, 78 to86), significantly lower than the 94% (95% CI,93 to 95) for cT3 non-DIRcases (p<0.001). Coxproportional hazards regression returned a higherrisk of metastatic death for DIR cT3 comparedwith non-DIRcT3 (HR, 3.3; 95% CI, 1.8 to 5.9;p<0.001). High-riskpathological features weremore frequent in DIR (41% vs 28%, p=0.004).There was no association between DIR and localtreatment failure.Conclusions Approximately 1 in 20 patients withretinoblastoma had DIR, which often presentedwith glaucoma, anterior segment involvement orintraocular bleeding. DIR was more lethal thannon-DIRcT3, with enucleation revealing high-riskpathology.