Yanagihara, Ryan T;
Takayesu, Jamie SK;
Crotty, Erin E;
Ji, Xunda;
Zou, Yihua;
Sultana, Sadia;
Rashid, Riffat;
... Stacey, Andrew W; + view all
(2025)
Clinical stage of retinoblastoma based on presenting signs of 4,578 patients from 121 countries.
Ophthalmology
10.1016/j.ophtha.2025.04.030.
(In press).
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Abstract
PURPOSE: To investigate the associations between presenting signs and clinical stage of retinoblastoma at diagnosis in a global sample of retinoblastoma patients. DESIGN: Cross-sectional analysis. SUBJECTS: Treatment-naive children diagnosed at 259 retinoblastoma referral centers across 121 countries during 2017 and 2019. METHODS: Presenting characteristics of patients incidentally diagnosed with retinoblastoma in 2017 and 2019 from two cross-sectional studies were analyzed. We examined the associations between presenting clinical sign (leukocoria, strabismus, orbital mass, orbital cellulitis, red eye, iris color change, and hyphema) and presenting stage of retinoblastoma. MAIN OUTCOME MEASURES: Frequency of clinical (cTNMH) and pathological (pTNM) stage per the American Joint Committee on Cancer Staging Manual Eighth Edition; extraocular stage per the International Retinoblastoma Staging System (IRSS), and intraocular classification at presentation. RESULTS: A total of 4,578 patients with retinoblastoma were included, 54.4% were male and the median presenting age was 23.1 months. Patients presenting with orbital mass and orbital cellulitis tended to present at later stages compared to those with leukocoria and strabismus. All signs, except for orbital mass, were more commonly seen in patients with curable disease (IRSS Stage 0/I/II): strabismus 94.8%, leukocoria 90.1%, iris changes 89.7%, hyphema 84.2%, red eye 82.1%, orbital cellulitis 66.7%, orbital mass 33.7%. Leukocoria was the most common presenting sign regardless of national income level, and the second most common sign was strabismus in high income countries and orbital mass in low-income countries. CONCLUSIONS: Any child with unexplained orbital cellulitis, persistent red eye, unexplained hyphema, orbital mass, or other changes in the iris or anterior chamber of the eye should undergo dilated fundus examination to rule out retinoblastoma. Prompt referral can lead to presentation with curable disease for all presenting signs.
| Type: | Article |
|---|---|
| Title: | Clinical stage of retinoblastoma based on presenting signs of 4,578 patients from 121 countries |
| Location: | United States |
| DOI: | 10.1016/j.ophtha.2025.04.030 |
| Publisher version: | https://doi.org/10.1016/j.ophtha.2025.04.030 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | cancer, cancer staging, Retinoblastoma, TNM classification |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10208481 |
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