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| | __NOTOC__ |
| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{DiseaseDisorder infobox |
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| Name = {{PAGENAME}} |
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| ICD10 = {{ICD10|C|69|2|c|69}} |
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| ICD9 = {{ICD9|190.5}} |
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| ICDO = {{ICDO|9510|3}} |
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| Image = Retinoblastoma.jpg |
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| Caption = Leukocoria of right eye indicating presence of tumor |
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| OMIM = 180200 |
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| MedlinePlus = |
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| eMedicineSubj = |
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| eMedicineTopic = |
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| DiseasesDB = 11434 |
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| MeshID = D012175 |
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| }}
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| {{Retinoblastoma}} | | {{Retinoblastoma}} |
| {{CMG}} | | {{CMG}}; {{AE}} {{Sahar}} {{Simrat}} |
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| {{Editor Join}} | | {{SK}} Retinoblastomas; RB; Rb |
| | ==[[Retinoblastoma overview|Overview]]== |
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| ==Overview== | | ==[[Retinoblastoma historical perspective|Historical Perspective]]== |
| '''Retinoblastoma''' is a [[cancer]] of the [[retina]]. Development of this tumor is initiated by [[mutation]]s<ref>{{cite journal |author=Knudson A |title=Mutation and cancer: statistical study of retinoblastoma |journal=Proc Natl Acad Sci U S A |volume=68 |issue=4 |pages=820-3 |year=1971 |pmid=5279gadgqetqer523}}</ref> that inactivate both copies of the ''[[RB1]]'' gene, which codes for the [[retinoblastoma protein]].<ref>{{cite journal |author=Friend S, Bernards R, Rogelj S, Weinberg R, Rapaport J, Albert D, Dryja T |title=A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma |journal=Nature |volume=323 |issue=6089 |pages=643-6 |year= |pmid=2877398}}</ref>
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| It occurs mostly in children younger than 5 years and accounts for about 3% of the cancers occurring in children younger than 15 years. Adult cases have also been clinically recorded.<ref>{{cite journal |author=Takahashi T, Tamura S, Inoue M, Isayama Y, Sashikata T |title=Retinoblastoma in a 26-year-old adult |journal=Ophthalmology |volume=90 |issue=2 |pages=179-83 |year=1983 |pmid=6856254}}</ref> The estimated annual incidence is approximately 4 per million children.<ref>[http://www.cancer.org/docroot/CRI/content/CRI_2_4_1X_What_are_the_key_statistics_for_retinoblastoma_37.asp?sitearea= cancer.org]</ref> It begins with white blotches in one or both eyes ([[leukocoria]]) which can be seen in photographs (this is distinct from the [[red-eye effect]] which is normal); or when light reflects off the eye, as when watching television.
| | ==[[Retinoblastoma classification #Retinoblastoma staging|Classification]]== |
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| The tumor may begin in one or both eyes. Retinoblastoma is usually confined to the eye but can spread to the brain via the [[optic nerve]].
| | ==[[Retinoblastoma pathophysiology|Pathophysiology]]== |
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| As the retina is the light-sensitive part of the eye necessary for vision, loss of vision occurs.
| | ==[[Retinoblastoma causes|Causes of Retinoblastoma]]== |
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| The condition reached the headlines in May 2007, when Utah Jazz point guard Derek Fisher revealed that his 10-month-old daughter had the condition. Fisher missed Game 1 and half of Game 2 of the 2007 NBA playoff series versus the Golden State Warriors to be with his daughter for her surgery in New York City.
| | ==[[Retinoblastoma differential diagnosis|Differentiating Retinoblastoma from other Diseases]]== |
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| ==Treatment== | | ==[[Retinoblastoma epidemiology and demographics|Epidemiology and Demographics]]== |
| Until recently the only treatment was to remove the affected eyeball before the cancer spread. Chemotherapy is the treatment of choice for most unilateral cases. However with locally advanced disease external beam radiation may be needed and if both eyes are involved [[enucleation]] may be the only option. Affected children in developing countries present with advanced features and usually die of metastatic spread. In its initial stages, retinoblastoma is very similar to [[Coats disease]], a non-cancerous retina disease. Coats' Disease should be ruled out before enucleation is done. A mis-diagnosis of Retinoblastoma accounts for the greatest number of Coats' disease eyes being enucleated.
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| Many children with bilateral retinoblastoma can be treated with a preservation attempt. Tumor [[chemotherapy|chemoreduction]] with [[carboplatin]] and other drugs may reduce the tumor volume making them amenable to local therapies <ref>{{cite journal |author=Dunkel IJ, Lee TC, Shi W, Beaverson KL, Novetsky D, Lyden D, Finlay JL, McCormick B, Abramson DH. |title=A phase II trial of carboplatin for intraocular retinoblastoma. |journal=Pediatr Blood Cancer. |pmid=17301956 }}</ref>
| | ==[[Retinoblastoma risk factors|Risk Factors]]== |
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| Local therapies include-
| | ==[[Retinoblastoma screening|Screening]]== |
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| [[Laser therapy]] (Uses infrared laser light to precisely destroy the blood vessels surrounding a tumor.) | | ==[[Retinoblastoma natural history, complications, and prognosis|Natural history, Complications, and Prognosis]]== |
| * [[Cryotherapy]] (use of a cold gas which is injected into the affected part of the retina to shrink the tumor.)
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| * [[Thermotherapy]] (A relatively new technique used mainly in new testing. It uses the principle that if heat is applied to the affected area, a tumor will sustain more damage than healthy cells because healthy cells can cool themselves better using healthy surrounding blood vessels. If this technique is not immediately successful it may increase the efficacy of other treatments such as chemotherapy and focused radiation plaques.)
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| * [[Radiotherapy]] (Generally used as a last resort, radiotherapy was previously the treatment of choice before the above mentioned treatments were developed. Radiotherapy destroys cancerous growths using [[gamma ray|gamma radiation]] but it carries with it many drawbacks, including:-
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| ** Possibility of secondary cancerous growths which present themselves months or years later.
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| ** Destruction of healthy cells in the area surrounding the treated tumor.
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| ** Bone deformation due to the destruction of the growth plates mainly in the area of the [[temple (anatomy)|temple]].)
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| It is important that children with retinoblastoma are treated in specialist centers.
| | ==Diagnosis== |
| | [[Retinoblastoma staging|Staging]] | [[Retinoblastoma history and symptoms|History & Symptoms]] | [[Retinoblastoma physical examination|Physical Examination]] | [[Retinoblastoma laboratory tests|Laboratory Tests]] | [[Retinoblastoma chest x ray|Chest X Ray]] | [[Retinoblastoma CT|CT]] | [[Retinoblastoma MRI|MRI]] | [[Retinoblastoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Retinoblastoma other imaging findings|Other Imaging Findings]] | [[Retinoblastoma other diagnostic studies| Other Diagnostic Studies]] |
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| [[Brachytherapy]] with [[beta ray|beta]]-emitting eye applicators have also been a successful major treatment. BEBIG (GmbH-Berlin-Germany) produces various kinds of [[ruthenium]] ophthalmic applicators for treating retinoblastoma. | | ==Treatment== |
| | | [[Retinoblastoma medical therapy|Medical Therapy]] | [[Retinoblastoma surgery|Surgery]] | [[Retinoblastoma primary prevention|Primary Prevention]] | [[Retinoblastoma secondary prevention|Secondary Prevention]] | [[Retinoblastoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Retinoblastoma future or investigational therapies|Future or Investigational Therapies]] |
| See Ben Underwood for a case who compensated for the resulting blindness by developing [[human echolocation]].
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| In October 2007, researchers have identified the specific cell that causes retinoblastoma.[http://www.sciencedaily.com/releases/2007/10/071018123509.htm]
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| ==References== | | ==References== |
| <references/>
| | {{reflist|2}} |
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| ==See also== | | ==See also== |
| *[[Eye cancer]] | | *[[Eye cancer]] |
| *[[Eye examination]] | | *[[Eye examination]] |
| | | [[Category:Medicine]] |
| ==External links==
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| *[http://www.cancer.gov/cancer_information/cancer_type/retinoblastoma/ The National Cancer Institute's page on Retinoblastoma]
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| *[http://www.retinoblastoma.net/ Retinoblastoma International]
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| * {{GeneTests|retinoblastoma}}
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| *[http://rb1-lsdb.d-lohmann.de/ Retinoblastoma Genetics]
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| *[http://bjo.bmj.com/cgi/content/full/84/10/1170/ At What Age Could Screening Be Stopped]
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| * [http://archopht.ama-assn.org/cgi/content/abstract/122/9/1324/ Visual Fields in Retinoblastoma Survivors]
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| * [http://www.mskcc.org/mskcc/html/62459.cfm Building on a Legacy of Innovation and Collaboration: Better Treatments for Retinoblastoma]
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| * {{cite journal |author=Abramson D, Frank C |title=Second nonocular tumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk |journal=Ophthalmology |volume=105 |issue=4 |pages=573-9; discussion 579-80 |year=1998 |pmid=9544627}}
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| {{Nervous tissue tumors}}
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| [[Category:Ophthalmology]]
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| [[Category:Types of cancer]]
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| [[Category:hereditary cancers]] | |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| | [[Category:Up-To-Date]] |
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| | | [[Category:Surgery]] |
| [[de:Retinoblastom]] | |
| [[es:Retinoblastoma]]
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| [[fr:Rétinoblastome]]
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| [[nl:Retinoblastoom]]
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| [[ja:網膜芽細胞腫]]
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| [[pl:Siatkówczak]]
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| [[pt:Retinoblastoma]]
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| [[ru:Ретинобластома]]
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| [[fi:Retinoblastooma]]
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| [[ur:شبکی ورمِ ارومہ]]
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