retinoblastoma
Pronunciation: ret-ih-no-blas-TO-mah
The most common primary intraocular malignancy in children, arising from neural retinal cells.
Full Definition
Retinoblastoma is a malignant tumor that develops from immature retinal cells and represents the most frequent primary intraocular cancer in pediatric patients. It typically manifests in children under 5 years of age and can be hereditary (germline mutations in RB1 gene) or sporadic. The classic presentation includes leukocoria (white pupillary reflex) and may involve one or both eyes. Early diagnosis and treatment are essential to preserve life, vision, and prevent secondary cancers in hereditary cases.
Usage
Usage note: Capitalize when referring to the International Classification of Retinoblastoma system.
In Context
- "Bilateral retinoblastoma was diagnosed at 18 months of age following genetic counseling." — Pediatric oncology case report
- "The International Classification of Retinoblastoma guides treatment decisions." — Treatment protocol document