Retinoblastoma

Retinoblastoma

Definition

A malignant tumor that originates in the nuclear layer of the retina. As the most common primary tumor of the eye in children, retinoblastoma is still relatively uncommon, accounting for only 1% of all malignant tumors in pediatric patients. Approximately 95% of cases are diagnosed before age 5. These tumors may be multifocal, bilateral, congenital, inherited, or acquired. Seventy-five percent of retinoblastomas are unilateral; 60% occur sporadically. A predisposition to retinoblastoma has been associated with 13q14 cytogenetic abnormalities. Patients with the inherited form also appear to be at increased risk for secondary nonocular malignancies such as osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma.

Also known as RB, retinoblastoma, retinoblastoma, malignant — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Camera-type eye Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Central nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Nervous system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Retina Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Retina Disease Has Primary Anatomic Site NCIT · CC BY 4.0

Drugs indicated

Drug Relation Source
Cyclophosphamide may treat MEDRT · Public domain (U.S. Government work)
Dactinomycin may treat MEDRT · Public domain (U.S. Government work)