Neoplastic Process
retinoblastoma
ret·ino·blas·to·ma [ ret-noh-bla-stoh-muh ]
Subclass of:
Neoplasms, Neuroepithelial;
Eye Diseases, Hereditary;
Retinal Neoplasms
Definitions related to retinoblastoma:
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(retinoblastoma, malignant) A malignant neoplasm originating in the nuclear layer of the retina.CDISC TerminologyClinical Data Interchange Standards Consortium (CDISC), 2021
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A malignant tumor arising from the nuclear layer of the retina that is the most common primary tumor of the eye in children. The tumor tends to occur in early childhood or infancy and may be present at birth. The majority are sporadic, but the condition may be transmitted as an autosomal dominant trait. Histologic features include dense cellularity, small round polygonal cells, and areas of calcification and necrosis. An abnormal pupil reflex (leukokoria); NYSTAGMUS, PATHOLOGIC; STRABISMUS; and visual loss represent common clinical characteristics of this condition. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2104)NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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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.NCI ThesaurusU.S. National Cancer Institute, 2021
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A tumor of the eye originating from cells of the retina.Human Phenotype Ontology (HPO)The Human Phenotype Ontology Project, 2021
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Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).NCI Dictionary of Cancer TermsU.S. National Cancer Institute, 2021
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Malignant congenital blastoma, occurring in both hereditary and sporadic forms, composed of tumor cells arising from the retinoblasts; the most common tumor of the eye in children.CRISP ThesaurusNational Institutes of Health, 2006
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Retinoblastoma is a malignant tumor of the developing retina that occurs in children, usually before age five years. Retinoblastoma develops from cells that have cancer-predisposing variants in both copies of RB1. Retinoblastoma may be unifocal or multifocal. About 60% of affected individuals have unilateral retinoblastoma with a mean age of diagnosis of 24 months; about 40% have bilateral retinoblastoma with a mean age of diagnosis of 15 months. Heritable retinoblastoma is an autosomal dominant susceptibility for retinoblastoma. Individuals with heritable retinoblastoma are also at increased risk of developing non-ocular tumors.GeneReviewsUniversity of Washington, 2021
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(retinal neuroblastoma) A neuroblastoma arising from the retina.NCI ThesaurusU.S. National Cancer Institute, 2021
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Retinoblastoma is a cancer arising from the immature retina. Symptoms and signs commonly include leukocoria (a white reflex in the pupil), strabismus, and, less often, inflammation and impaired vision. Diagnosis is based on ophthalmoscopic examination and ultrasonography, CT, or MRI. Treatment of small cancers and bilateral disease may...Merck & Co., Inc., 2020
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The most widely held concept of histogenesis of retinoblastoma holds that it generally arises from a multipotential precursor cell (mutation in the long arm of chromosome 13 band 13q14) that could develop into almost any type of inner or outer retinal cell. Intraocularly, it exhibits a variety of growth patterns, which have been...WebMD, 2019
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Retinoblastoma is an eye cancer that begins in the retina - the sensitive lining on the inside of your eye. Retinoblastoma most commonly affects young children, but can rarely occur in adults.Mayo Foundation for Medical Education and Research
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Retinoblastoma (RB) is a rare type of eye cancer in the retina that typically develops before the age of 5. It usually affects only one eye, but 1/3 of children with RB develop cancer in both eyes. The first sign is typically a visible whiteness in the pupil called "cat's eye reflex" or leukocoria, which is particularly noticeable in...National Center for Advancing Translational Sciences
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The most common malignant intraocular tumor in children. It can be unilateral or bilateral, familial or spontaneous, and in 30% to 40% of cases is accompanied by a germinal mutation in the RB1 gene. It often causes an exudative retinal detachment with vitreous seeding.Athenahealth, Inc., 2019
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