High-risk Pathologic Features Based on Presenting Findings in Advanced Intraocular Retinoblastoma: A Multicenter,...

Area of Science:

• Ophthalmology
• Oncology
• Pathology

Background:

• Retinoblastoma is the most common intraocular malignancy in children.
• Accurate staging is crucial for treatment planning and predicting outcomes.
• The American Joint Committee on Cancer (AJCC) staging system aids in classifying retinoblastoma severity.

Purpose of the Study:

• To evaluate the predictive value of clinical features within the AJCC cT3 category for advanced intraocular retinoblastoma.
• To assess the utility of AJCC Ophthalmic Oncology Task Force (OOTF) Size Groups in identifying high-risk pathologic features.
• To determine if specific clinical indicators can reliably predict high-risk pathological findings in retinoblastoma.

Main Methods:

• A retrospective, international, multicenter registry-based study.
• Data from 942 eyes enucleated for AJCC cT3 and cT2 retinoblastoma between 2001 and 2013 were analyzed.
• High-risk features were defined as AJCC pT3/pT4; AJCC OOTF Size Groups (1-4) categorized tumor volume.

Main Results:

• Of 942 eyes, 282 (30%) exhibited high-risk pathologic features.
• AJCC cT3 subcategories (cT3c-e: glaucoma, hemorrhage, cellulitis) and AJCC OOTF Size Groups 3-4 (large/diffuse tumors) were significantly associated with high-risk features (P < 0.001).
• Odds ratios for high-risk features were elevated for cT3c (2.3), cT3d (2.5), cT3e (3.3), Size Group 3 (3.3), and Size Group 4 (4.1) compared to controls.

Conclusions:

• Clinical subcategories cT3c-e (glaucoma, intraocular hemorrhage, aseptic orbital cellulitis) are valuable predictors of high-risk pathologic features in retinoblastoma.
• AJCC OOTF Size Groups 3 (tumor > 2/3 globe volume) and 4 (diffuse infiltrative retinoblastoma) effectively stratify risk for high-risk pathologic features.
• These clinical factors aid in predicting high-risk pathology, facilitating informed treatment decisions for retinoblastoma.