retinoblastoma
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.
MONDO:0015966
obsolete hereditary eye tumor (obsolete MONDO:0015966)
excluded_subClassOf
MONDO:0020238
obsolete inherited vitreous-retinal disease (obsolete MONDO:0020238)
excluded_subClassOf
MONDO:0004338
retinal cell cancer
is_a
RB
exact
RB1
narrow
eye cancer, retinoblastoma
related
retinoblastoma
exact
retinoblastoma, malignant
exact