Diffuse infiltrating retinoblastoma: a multicentre, international, data-sharing study
Por:
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, Català-Mora J, Correa-Llano, G, Carreras-Bertran E and AJCC OOTF
Publicada:
1 dic 2025
Ahead of Print:
1 jul 2025
Resumen:
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.
Filiaciones:
Tomar, AS:
New York Eye Canc Ctr, Ocular Tumor Orbital Dis & Ophtham Radiat Therapy, New York, NY 10065 USA
Finger, PT:
New York Eye Canc Ctr, Ocular Tumor Orbital Dis & Ophtham Radiat Therapy, New York, NY 10065 USA
Gallie, B:
Hosp Sick Children, Dept Ophthalmol & Vis Sci, Retinoblastoma Program, Toronto, ON, Canada
Kivelä, TT:
Univ Helsinki, Dept Ophthalmol, Ocular Oncol Serv, Cent Hosp, Helsinki, Uusimaa, Finland
Mallipatna, A:
Hosp Sick Children, Dept Ophthalmol & Vis Sci, Retinoblastoma Program, Toronto, ON, Canada
Narayana Nethralaya Eye Hosp, Dept Ocular Oncol, Bangalore, India
Zhang, CY:
Beijing Childrens Hosp, Pediat Oncol Ctr, Dept Ophthalmol, Beijing, Peoples R China
Zhao, JY:
Beijing Childrens Hosp, Pediat Oncol Ctr, Dept Ophthalmol, Beijing, Peoples R China
Wilson, MW:
Hamilton Eye Inst, Dept Ophthalm Oncol, Memphis, TN USA
Brennan, RC:
St Jude Childrens Res Hosp, Ophthalmol Div, Memphis, TN USA
Burges, M:
Coll Med, Dept Ophthalmol, Memphis, TN USA
Berry, JL:
Childrens Hosp Los Angeles, Doheny Eye Inst, Ocular Oncol & Retinoblastoma, Los Angeles, CA USA
Jubran, R:
Childrens Hosp Los Angeles, Canc & Blood Dis, Los Angeles, CA USA
Khetan, V:
Sankara Nethralaya, Ocular Oncol & Vitreoretina, Chennai, Tamil Nadu, India
Ganesan, S:
Sankara Nethralaya, Ocular Oncol & Vitreoretina, Chennai, Tamil Nadu, India
Yarovoy, A:
SN Fyodorov Eye Microsurg Fed State Inst, Ocular Oncol Dept, Moscow, Russia
Yarovaya, V:
SN Fyodorov Eye Microsurg Fed State Inst, Ocular Oncol Dept, Moscow, Russia
Kotova, E:
SN Fyodorov Eye Microsurg Fed State Inst, Ocular Oncol Dept, Moscow, Russia
Volodin, D:
SN Fyodorov Eye Microsurg Fed State Inst, Ocular Oncol Dept, Moscow, Russia
Yousef, YA:
King Hussein Canc Ctr, Surg Ophthalmol, Amman, Jordan
Nummi, K:
Univ Helsinki, Dept Ophthalmol, Ocular Oncol Serv, Cent Hosp, Helsinki, Uusimaa, Finland
Ushakova, TL:
Med Acad Postgrad Educ, Pediat Oncol, Moscow, Russia
NN Blokhin Natl Med Res Ctr Oncol Russian Federat, SRI Pediat Oncol & Hematol, Moscow, Russia
Yugay, O:
NN Blokhin Natl Med Res Ctr Oncol Russian Federat, SRI Pediat Oncol & Hematol, Moscow, Russia
Polyakov, VG:
Med Acad Postgrad Educ, Pediat Oncol, Moscow, Russia
NN Blokhin Natl Med Res Ctr Oncol Russian Federat, SRI Pediat Oncol & Hematol, Moscow, Russia
Ramirez-Ortiz, MA:
Hosp Infantil Mexico Dr Federico Gomez, Dept Ophthalmol, Mexico City, Mexico
Esparza-Aguiar, E:
Hosp Infantil Mexico Dr Federico Gomez, Dept Ophthalmol, Mexico City, Mexico
Chantada G:
Hosp JP Garrahan, Hematooncol, Buenos Aires, Argentina
St Joan de Deu Hosp, Oncol, Barcelona, Barcelona, Spain
Schaiquevich, P:
Hosp Pediat JP Garrahan, Buenos Aires, Argentina
Fandiño, A:
Hosp Pediat JP Garrahan, Buenos Aires, Argentina
Yam, JC:
Hong Kong Eye Hosp, Dept Ophthalmol & Visual Sci, Hong Kong, Peoples R China
Lau, WW:
Hong Kong Eye Hosp, Dept Ophthalmol & Visual Sci, Hong Kong, Peoples R China
Lam, CP:
Hong Kong Eye Hosp, Dept Ophthalmol & Visual Sci, Hong Kong, Peoples R China
Sharwood, P:
Univ Sydney, Sydney, NSW, Australia
Moorthy, S:
KK Womens & Childrens Hosp, Singapore, Singapore
Long, QB:
KK Womens & Childrens Hosp, Singapore, Singapore
Essuman, VA:
Univ Ghana, Univ Ghana Med Sch, Coll Hlth Sci, Accra, Ghana
Renner, LA:
Univ Ghana, Dept Child Hlth, Coll Hlth Sci, Accra, Ghana
Semenova, E:
New York Eye Canc Ctr, Ocular Tumor Orbital Dis & Ophtham Radiat Therapy, New York, NY 10065 USA
Català-Mora J:
Hosp Univ St Joan de Deu, Ophthalmol, Barcelona, Spain
:
St Joan de Deu Hosp, Oncol, Barcelona, Barcelona, Spain
Carreras-Bertran E:
Hosp Univ St Joan de Deu, Ophthalmol, Barcelona, Spain
AJCC OOTF:
Amer Joint Comm Canc, Chicago, IL USA
Green Submitted
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