Retinoblastoma


Retinoblastoma
A malignant eye tumor caused by the loss of a pair of tumor-suppressor genes. An inherited form of retinoblastoma (it typically appears at birth, leads to multiple tumors and affects both eyes) is due to a transmissible (germline) mutation followed by an acquired (somatic) mutation. The sporadic form of retinoblastoma (it has later onset and leads to a single tumor in one eye) is due to acquired (somatic) mutations of both tumor-suppressor genes. When the tumor is detected at an early stage, it can sometimes be treated locally, but it often unfortunately requires removal of the eye (enucleation).
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Malignant ocular neoplasm of childhood, with onset usually before the third year of life, composed of primitive retinal small round cells with deeply staining nuclei and elongated cells forming rosettes; there is an increased risk of developing osteosarcoma later in life. In familial cases, the disease is usually bilateral with multiple lesions within an eye, but in sporadic cases rarely so. Autosomal dominant inheritance caused by mutation in the tumor-suppressor r. gene (RB) on chromosome 13q. [retino- + G. blastos, germ, + -oma, tumor]

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ret·i·no·blas·to·ma .ret-ən-ō-.blas-'tō-mə n, pl -mas also -ma·ta -mət-ə a hereditary malignant tumor of the retina that develops during childhood, is derived from retinal germ cells, and is associated with a chromosomal abnormality

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n.
a rare malignant tumour of the retina, occurring in infants.

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ret·i·no·blas·to·ma (ret″ĭ-no-blas-toґmə) [retina + blastoma] a malignant, often congenital blastoma, occurring in both hereditary and sporadic forms, composed of tumor cells arising from embryonic retinal cells. it appears in one or both eyes in children under 5 years of age, and is usually diagnosed initially by a bright white or yellow pupillary reflex (leukokoria). It is caused by germline or somatic mutations, or both, in both alleles of the retinoblastoma (RB1) gene. Called also glioma retinae.

Medical dictionary. 2011.

Look at other dictionaries:

  • Retinoblastoma — Saltar a navegación, búsqueda Retinoblastoma Clasificación y recursos externos Aviso médico …   Wikipedia Español

  • retinoblastoma — m. oftalm. Tumor maligno que se desarrolla en las células retinianas. Generalmente es bilateral y hereditario de transmisión autosómica dominante. Suele descubrirse en los niños a una edad comprendida entre los 2 y los 4 años por la aparición de… …   Diccionario médico

  • Retinoblastoma — es un cáncer de la retina. Es causado por una mutación en la proteína Rb 1. Se presenta en mayor parte en niños pequeños y representa el 3% de los cánceres padecidos por menores de quince años. La incidencia anual estimada es de aproximadamente 4 …   Enciclopedia Universal

  • retinoblastoma — retinoblastoma. См. ретинобластома. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • retinoblastoma — [ret΄ n ō blas tō′mə] n. pl. retinoblastomas, retinoblastomata [ret΄ n ō blas tō′mə tə] a rare, often inherited, malignant tumor that forms in the retina, usually of a young child …   English World dictionary

  • Retinoblastoma — DiseaseDisorder infobox Name = PAGENAME ICD10 = ICD10|C|69|2|c|69 ICD9 = ICD9|190.5 ICDO = ICDO|9510|3 Caption = Leukocoria of right eye indicating presence of tumor OMIM = 180200 MedlinePlus = 001030 eMedicineSubj = oph eMedicineTopic = 346… …   Wikipedia

  • retinoblastoma — Malignant tumour of the retina, usually arising in the inner nuclear layer of the neural retina. Retinoblastoma is unusual in being caused by an autosomal dominant mutation in some cases (about 6%), in which case it may be bilateral. The gene… …   Dictionary of molecular biology

  • retinoblastoma — Eng. Retinoblastoma Tumor primario, congénito y maligno constituido por células de la retina neurosensoriales precursoras de los fotorreceptores …   Diccionario de oftalmología

  • retinoblastoma — {{#}}{{LM R34122}}{{〓}} {{[}}retinoblastoma{{]}} ‹re·ti·no·blas·to·ma› {{《}}▍ s.m.{{》}} Tumor maligno que se desarrolla en la retina …   Diccionario de uso del español actual con sinónimos y antónimos

  • retinoblastoma — 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) …   English dictionary of cancer terms


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