Preferred Name | Retinoblastoma | |
Synonyms |
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Definitions |
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. |
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ID |
http://purl.obolibrary.org/obo/NCIT_C7541 |
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ALT_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. 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). A malignant neoplasm originating in the nuclear layer of the retina. |
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code |
C7541 |
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Contributing_Source |
Cellosaurus CDISC CPTAC CTEP CTRP GDC MedDRA NICHD PCDC |
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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. |
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Display_Name |
Retinoblastoma |
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ICD-O-3_Code |
9510/3 |
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in_subset |
http://purl.obolibrary.org/obo/NCIT_C138190 http://purl.obolibrary.org/obo/NCIT_C168658 http://purl.obolibrary.org/obo/NCIT_C168656 http://purl.obolibrary.org/obo/NCIT_C168657 http://purl.obolibrary.org/obo/NCIT_C168655 http://purl.obolibrary.org/obo/NCIT_C77526 http://purl.obolibrary.org/obo/NCIT_C90259 http://purl.obolibrary.org/obo/NCIT_C158520 http://purl.obolibrary.org/obo/NCIT_C179491 http://purl.obolibrary.org/obo/NCIT_C102905 http://purl.obolibrary.org/obo/NCIT_C168662 http://purl.obolibrary.org/obo/NCIT_C168661 http://purl.obolibrary.org/obo/NCIT_C177537 http://purl.obolibrary.org/obo/ncit#oncotree_slim http://purl.obolibrary.org/obo/NCIT_C156952 http://purl.obolibrary.org/obo/NCIT_C116977 http://purl.obolibrary.org/obo/NCIT_C118464 http://purl.obolibrary.org/obo/NCIT_C132009 http://purl.obolibrary.org/obo/NCIT_C126659 http://purl.obolibrary.org/obo/NCIT_C138188 http://purl.obolibrary.org/obo/NCIT_C179478 http://purl.obolibrary.org/obo/NCIT_C88025 http://purl.obolibrary.org/obo/NCIT_C165258 http://purl.obolibrary.org/obo/NCIT_C103061 http://purl.obolibrary.org/obo/NCIT_C118168 http://purl.obolibrary.org/obo/NCIT_C157711 http://purl.obolibrary.org/obo/NCIT_C156966 |
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Is_Value_For_GDC_Property | ||
label |
Retinoblastoma |
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Legacy Concept Name |
Retinoblastoma |
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Maps_To |
Retinoblastoma Retinoblastoma, NOS 9510/3 |
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Neoplasm_Has_Special_Category | ||
Neoplastic_Status |
Malignant |
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Preferred_Name |
Retinoblastoma |
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prefixIRI |
NCIT:C7541 |
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prefLabel |
Retinoblastoma |
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Semantic_Type |
Neoplastic Process |
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UMLS_CUI |
C0035335 |
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subClassOf |
http://purl.obolibrary.org/obo/NCIT_C7061 |