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Publications

Journal Article Ahead of print 1 citation

PubMed: 40738737 DOI: 10.1136/bjo-2024-326778

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

Br J Ophthalmol

Authors: Ankit Singh Tomar, Paul T Finger, Brenda Gallie, Tero T Kivelä, Ashwin Mallipatna, Chengyue Zhang, Junyang Zhao, Matthew W Wilson, Rachel C Brennan, Michala Burges, Jesse L Berry, Rima Jubran, Vikas Khetan, Suganeswari Ganesan, Andrey Yarovoy, Vera Yarovaya, Elena Kotova, Denis Volodin, Yacoub A Yousef, Kalle Nummi, Tatiana L Ushakova, Olga V Yugay, Vladimir G Polyakov, Marco A Ramirez-Ortiz, Elizabeth Esparza-Aguiar, Guillermo Chantada, Paula Schaiquevich, Adriana Fandiño, Jason C Yam, Winnie W Lau, Carol P Lam, Phillipa Sharwood, Sonia Moorthy, Quah Boon Long, Vera Adobea Essuman, Lorna A Renner, Ekaterina Semenova, Jaume Catala-Mora, Genoveva Correa-Llano, Elisa Carreras

Affiliations

  • Ocular Tumor, Orbital Disease, and Ophthamic Radiation Therapy, The New York Eye Cancer Center, New York, New York, USA.
  • Ocular Tumor, Orbital Disease, and Ophthamic Radiation Therapy, The New York Eye Cancer Center, New York, New York, USA.
  • Department of Ophthalmology and Vision Sciences, Retinoblastoma Program, Hospital for Sick Children, Toronto, Ontario, Canada.
  • Ocular Oncology Service, Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Uusimaa, Finland.
  • Department of Ocular Oncology, Narayana Nethralaya Eye Hospital, Bangalore, India.
  • Department of Ophthalmology, Pediatric Oncology Center, Beijing Children's Hospital, Beijing, China.
  • Department of Ophthalmic Oncology, The Hamilton Eye Institute, Memphis, Tennessee, USA.
  • Ophthalmology Division, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
  • Department of Ophthalmology, College of Medicine, Memphis, Tennessee, USA.
  • Ocular Oncology and Retinoblastoma, Doheny Eye Institute, Children's Hospital Los Angeles, Los Angeles, California, USA.
  • Cancer and Blood Diseases, Children's Hospital Los Angeles, los Angeles, California, USA.
  • Ocular Oncology and VitreoRetina, Sankara Nethralaya, Chennai, Tamil Nadu, India.
  • Ocular Oncology Department, The SN Fyodorov Eye Microsurgery Federal State Institution, Moscow, Russian Federation.
  • Surgery (Ophthalmology), King Hussein Cancer Center, Amman, Jordan.
  • Pediatric Oncology, Medical Academy of Postgraduate Education, Moscow, Russian Federation.
  • SRI of Pediatric Oncology and Hematology of NN Blokhin National Medical Research Center Oncology of Russian Federation, Moscow, Russian Federation.
  • Department of Ophthalmology, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico.
  • Hematooncology, Hospital JP Garrahan, Buenos Aires, Argentina, Buenos Aires, Argentina.
  • Oncology, Sant Joan de Deu Hospital, Barcelona, Barcelona, Spain.
  • Hospital de Pediatria JP Garrahan, Buenos Aires, Buenos Aires, Argentina.
  • Department of Ophthalmology and Visual Sciences, Hong Kong Eye Hospital, Hong Kong, Hong Kong.
  • University of Sydney, Sydney, New South Wales, Australia.
  • KK Women's and Children's Hospital, Singapore.
  • University of Ghana Medical School, College of Health Sciences, University of Ghana, Accra, Ghana.
  • Department of Child Health, College of Health Sciences University of Ghana, Accra, Ghana.
  • Ophthalmology, Hospital Universitari Sant Joan de Déu, Barcelona, Spain.

Abstract

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.

MeSH

Eye, Retinoblastoma, Ophthalmology, Glaucoma, Therapeutics