Diffuse infiltrating retinoblastoma: a multicentre, international, data-sharing study

Background To determine the unique clinical characteristics and treatment outcomes of diffuse infiltrating retinoblastoma (DIR). Methods This international, multicentre, registry-based retrospective case series analysed pooled data from January 2001 to December 2013, including 132 eyes from 132 pati...

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Autores: Tomar, AS; Finger, PT; Gallie, B; Kivelä, TT; Mallipatna, A; Zhang, CY; Zhao, JY; Wilson, MW; Brennan, RC; Burges, M; Berry, JL; Jubran, R; Khetan, V; Ganesan, S; Yarovoy, A; Yarovaya, V; Kotova, E; Volodin, D; Yousef, YA; Nummi, K; Ushakova, TL; Yugay, O; Polyakov, VG; Ramirez-Ortiz, MA; Esparza-Aguiar, E; Chantada, G; Schaiquevich, P; Fandiño, A; Yam, JC; Lau, WW; Lam, CP; Sharwood, P; Moorthy, S; Long, QB; Essuman, VA; Renner, LA; Semenova, E; Catala-Mora, J; Correa-Llano, G; Carreras, E; AJCC OOTF
Tipo de documento: Artigo
Estado:Published version
Data de publicação:2025
País:España
Recursos:Fundació Sant Joan de Déu
Repositório:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p29335
Acesso em linha:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29335
Nível de acesso:Acesso aberto
Palavra-chave:Neoplasia
Pathology
Retina
Hemorrhage
Uveitis
Resumo:Background To determine the unique clinical characteristics and treatment outcomes of diffuse infiltrating retinoblastoma (DIR). Methods This international, multicentre, registry-based retrospective case series analysed pooled data from January 2001 to December 2013, including 132 eyes from 132 patients with DIR. Results Among 2854 eyes with retinoblastoma, 132 (4.6%; 95% CI, 3.9 to 5.5) had DIR. The median age at diagnosis for DIR patients was 24 months (IQR, 15-33), with no bilateral cases of DIR. The American Joint Committee on Cancer staging showed 4.5% cT2 and 95.5% cT3 categories, with no cT1 or cT4 cases. Clinical features associated with DIR included secondary glaucoma (67%), retinal detachment (38%), diffuse vitreous seeds (37%), anterior segment involvement (24%), vitreous haemorrhage (50%) and hyphema (6%). Primary enucleation was the predominant treatment (81%), while 19% initially received systemic chemotherapy, with 6% requiring subsequent enucleation. The 5-year Kaplan-Meier survival rate for cT3 DIR was 82% (95% CI, 78 to 86), significantly lower than the 94% (95% CI, 93 to 95) for cT3 non-DIR cases (p<0.001). Cox proportional hazards regression returned a higher risk of metastatic death for DIR cT3 compared with non-DIR cT3 (HR, 3.3; 95% CI, 1.8 to 5.9; p<0.001). High-risk pathological features were more frequent in DIR (41% vs 28%, p=0.004). There was no association between DIR and local treatment failure. Conclusions Approximately 1 in 20 patients with retinoblastoma had DIR, which often presented with glaucoma, anterior segment involvement or intraocular bleeding. DIR was more lethal than non-DIR cT3, with enucleation revealing high-risk pathology.

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