61. THE MERCK MANUAL, Sec. 19, Ch. 266, Neoplasms retinoblastoma. A malignant tumor that arises from the immature retina. retinoblastoma occurs in 1/15000 to 1/30000 live births and represents about 2% of http://www.merck.com/mrkshared/mmanual/section19/chapter266/266d.jsp

62. THE MERCK MANUAL--SECOND HOME EDITION, Retinoblastoma In Ch. 283, Childhood Canc Symptoms of a retinoblastoma can include a white pupil or strabismus Without treatment, most children with retinoblastoma die within 2 years. http://www.merck.com/mmhe/sec23/ch283/ch283d.html

var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Children's Health Issues Chapter Childhood Cancers Topics Introduction Neuroblastoma Retinoblastoma Wilms' Tumor Retinoblastoma Buy The Book Print This Topic Email This Topic Pronunciations anesthesia computed tomography cyclophosphamide melanoma ... strabismus Retinoblastoma is a cancer of the retina, the light-sensing area at the back of the eye. Retinoblastomas represent about 2% of childhood cancers and almost always occur before age 4. They occur in both eyes at the same time in 20 to 30% of children. This cancer is the result of damage to specific, known genes that control eye development. Sometimes a damaged gene is inherited from a parent or becomes damaged very early during embryonic development. Children with this type of damage may pass the defective gene on to their offspring, who may also develop retinoblastoma. Other times, genes are damaged later in embryonic development only in the embryo's eye cells. This damage cannot be passed on to offspring. Retinoblastoma is hereditary in all of the children with cancer in both eyes and in 15 to 20% of children with cancer in one eye.

63. Home At Solutions our goal is to identify the precise error in a gene that causes illness or cancer in a specific family. http://www.retinoblastomasolutions.org/

"... simpler, less costly and more precise heath care, directly and transparently linked to scientifically validated diagnostic technology." Quick Links What is Retinoblastoma? Who should be tested? How to submit samples? Home ... Site map

64. For Families At Solutions our goal is to identify the precise error in a gene that causes illness or cancer in a specific family. http://www.retinoblastomasolutions.org/families.html

About us Retinoblastoma gene testing Submit samples ... Links Information for Families Information about Retinoblastoma Organizations that support retinoblastoma families Online discussion groups Exceptional Explanations of Retinoblastoma A Parent's Guide to Understanding Retinoblastoma, DH Abramson, MD, FACS Retinoblastoma , P Finger, MD, FACS Sites with Additional Information about Retinoblastoma Oncolink, General Retinoblastoma Information Pediatric Oncology Resource Center, General Retinoblastoma Information The Retinoblastoma Gene Retinoblastoma Fact Sheet, Royal National Institute for the Blind (UK) ... North East Valley Division of General Practice General Retinoblastoma Information The Retinoblastoma Society (UK) Prosthetic Eye Institute St. Jude's Hospital, General Retinoblastoma Information Children's Cancer Web, Retinoblastoma Page ... HealthCentral, General Health Encyclopedia, Retinoblastoma General Retinoblastoma Information Sarah's Retinoblastoma Homepage Canadian organizations that support retinoblastoma families Canadian Retinoblastoma Society c/o 33 South Street Port Hope, ON L1A 1R9

65. Retinoblastoma Penn State Children s Hospital provides world class care and services to patients. http://www.hmc.psu.edu/childrens/healthinfo/r/retinoblastoma.htm

66. International Gathering To Share Retinoblastoma Breakthroughs Experts in the fields of retinoblastoma research and treatment will gather to update colleagues on the latest developments in these fields during a twoday http://www.stjude.org/media/0,2561,453_2816_17452,00.html

@import url(/StJude/CDA/Common/CSS/default.css); @import url(/StJude/CDA/Common/CSS/default_lists.css); @import url(/StJude/CDA/Common/CSS/default_content_types.css); @import url(/StJude/Common/CSS/St_Jude_News); In order for our site to display correctly you will need a newer version of your web browser. The following browsers support numerous web standards including CSS, XHTML, and the DOM (a universal means of controlling the behavior of web pages): v7 or higher (all platforms), Mozilla FireFox (Windows), Camino (Mac OS X), Galeon (Linux GNOME Desktop) or other browser stemming from the Mozilla.org project. Opera v7 or higher (Windows, Linux) Apple's Safari (Mac OS X) Konqueror (Linux KDE Desktop) Microsoft Internet Explorer v6 or higher (Windows) or v5 or higher (Macintosh) St. Jude Children's Research Hospital Finding Cures. Saving Children. About St. Jude News Donate Now Contact Us ... Ways to Help News Releases Archives Home News Home Media ... News Releases Related Topics Michael A. Dyer, PhD Carlos Rodriguez-Galindo, MD Barrett G. Haik, MD, FACS International gathering to share retinoblastoma breakthroughs Memphis, Tennessee, February 16, 2005

67. Retinoblastoma & Miracles Benefit Homepage Page written and maintained by the parent of a child with retinoblastoma. This retinoblastoma and Miracles Benefit site is owned by Sally A. Wilson http://remarque.org/~sally/Miracles.html

document.write(''); Visitors since May 26th, 1998 [Page updated (16:30 PST) Friday, December 1st, 2000] Welcome If anyone has suggestions for improvements, new links, or related articles please e-mail the the page; or if you have information on related legitimate charities or benefits that you would like to advertise on the page. Sally A. Wilson, Sally e-mail: sally@remarque.org GoTo.com please utilize it for your web.net searches. GoTo pays 3 cents for every search; I have set it up that the money that the page makes will be donated to Retinoblastoma International, Children's Hospital Los Angeles. Article, Inside Soap Magazine Article, Inside Soap Magazine Actor Matthew Ashford talks about dealing with a child with Rb and his activism for Rb Awareness. Information on how to help a Sacramento Rb Survivor: Below is information concerning the Sacramento Bee newspaper's Book of Dreams , and how you can help Christina Parra.

68. Retinoblastoma : St. Louis Children's Hospital Detailed information on retinoblastoma, including causes, stages, symptoms, diagnosis, and treatment. http://www.stlouischildrens.org/articles/content.asp?pageid=P02742

69. Retinoblastoma - General Practice Notebook retinoblastoma is a rare tumour of the eye affecting about 1 in 25 000 individuals. The majority are seen in children younger than 5 years. http://www.gpnotebook.co.uk/simplepage.cfm?ID=1248526346

70. RETINOBLASTOMA retinoblastoma. What is retinoblastoma? retinoblastoma is the commonest eye cancer in infancy and childhood, affecting one in seventeen thousand live births http://www.nevdgp.org.au/geninf/std_misc/RETINOBLASTOMA.htm

71. Retinoblastoma When retinoblastoma is bilateral, enucleation is done on the worst eye first, There is some evidence that children with retinoblastoma have better http://www.tsbvi.edu/Education/anomalies/retinoblastoma.htm

RETINOBLASTOMA DESCRIPTION: A malignant and life-threatening intraocular tumor which appears in children; 2/3rds appear before age 3, however, rare cases have been reported at almost every age. About 30% are bilateral. It usually is unnoticed until it has progressed to a point of producing a white pupil (unless it has caused a strabismus and is diagnosed earlier, since blind eyes in children will often turn inward). Generally, the earlier the tumor is discovered, the better the chance to treat it and prevent its spread through the optic nerve and orbital tissues. About 94% of these tumors are due to mutations, but survivors will pass the mutated gene on about 50% of the time. TREATMENT: Enucleation is the treatment of choice when the tumor is large; radiotherapy and/or chemotherapy are other possibilities. Occasionally, cryotherapy or photocoagulation are effective. When retinoblastoma is bilateral, enucleation is done on the worst eye first, with radiotherapy attempted on the other eye. If no improvement is observed, enucleation is done on the second eye. GENETIC COUNSELING IS ESSENTIAL.

72. InteliHealth: Retinoblastoma InteliHealth Featuring Harvard Medical School s consumer health information. For more than 550 diseases and conditions, learn What Is It?, Symptoms, http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/10614.html

chrome_imgPreload('gifChr_mid_but_home_mo_1','http://img.intelihealth.com/i/C/Chr_mid_but_home-o.gif'); chrome_imgPreload('gifChr_mid_but_comm_mo_2','http://img.intelihealth.com/i/C/Chr_mid_but_comm-o.gif'); chrome_imgPreload('gifChr_mid_but_dental_mo_3','http://img.intelihealth.com/i/C/Chr_mid_but_dental-o.gif'); chrome_imgPreload('gifChr_mid_but_drug_mo_4','http://img.intelihealth.com/i/C/Chr_mid_but_drug-o.gif'); chrome_imgPreload('gifChr_mid_but_askexpert_mo_5','http://img.intelihealth.com/i/C/Chr_mid_but_askexpert-o.gif'); chrome_imgPreload('gifChr_mid_but_medical_mo_6','http://img.intelihealth.com/i/C/Chr_mid_but_medical-o.gif'); chrome_imgPreload('gifChr_mid_but_chats_mo_7','http://img.intelihealth.com/i/C/Chr_mid_but_chats-o.gif'); chrome_imgPreload('gifchr_mid_but_news_mo_8','http://img.intelihealth.com/i/c/chr_mid_but_news-o.gif'); chrome_imgPreload('gifChr_mid_but_privacy_mo_9','http://img.intelihealth.com/i/C/Chr_mid_but_privacy-o.gif'); Advertisement Retinoblastoma What Is It? Symptoms Diagnosis Expected Duration ... Additional Info What Is It? Retinoblastoma, a cancerous tumor of the retina in the eye, is a rare form of cancer that affects children, most commonly before age 5. The retina is the nerve tissue at the back of the eye that senses light and sends images to the brain, which interprets these images.

73. Retinoblastoma Ophthalmology Hematology and Oncology Peds retinoblastoma. http://www.fpnotebook.com/EYE44.htm

Home About Links Index ... Editor's Choice document.write(code); Advertisement Ophthalmology Hematology and Oncology Pediatrics Retinoblastoma Retinoblastoma Book Home Page Cardiovascular Medicine Dentistry Dermatology Emergency Medicine Endocrinology Gastroenterology Geriatric Medicine Gynecology Hematology and Oncology HIV Infectious Disease Jokes Laboratory Neonatology Nephrology Neurology Obstetrics Ophthalmology Orthopedics Otolaryngology Pediatrics Pharmacology Practice Management Prevention Psychiatry Pulmonology Radiology Rheumatology Sports Medicine Surgery Urology Chapter Ophthalmology Index Anterior Conjunctival Disorders Corneal Disorders Cardiovascular Medicine Examination Glaucoma Hematology and Oncology Injury Lacrimal Lens Lid General Neurology Pediatrics Pharmacology Prevention Pupil Retina Rheumatology Sclera Sports Medicine Surgery Symptom Evaluation Vision Page Hematology and Oncology Index Peds Retinoblastoma Epidemiology Rare malignant tumor of retina Incidence : 1 in 20,000 live births Average age of diagnosis: 12-18 months old Possible hereditary etiology Pathophysiology Retinal germ cell tumor Tumor invades vitreous or grows beneath retina Signs: Screen at every well child check in first 2 years Pupillary Light Reflex by direct ophthalmoscope White ( Leukokoria ) replaces Red Reflex (60% of cases) Strabismus testing Management Enucleation (may not be required in early treatment) Radiation Laser photocoagulation Cryotherapy Systemic Chemotherapy Associated conditions Strabismus Osteogenic Sarcoma Prognosis Uniformly fatal without treatment Survival of primary tumor with treatment: 90%

74. Case Of The Month - January, 2004 retinoblastoma is usually confined to the eye with 90% of children with 5-year survival rate 90%. -Extraocular involvement associated with 5-year survival http://cases.pedrad.org/case.php?month=January&year=2004

75. Retinoblastoma Definition retinoblastoma A cancer of the human retina. Predisposition to retinoblastoma is inherited as an autosomal dominant. A childhood cancer of retinoblast http://www.biochem.northwestern.edu/holmgren/Glossary/Definitions/Def-R/retinobl

Genes / Proteins Definitions Models Developmental Models ... Z retinoblastoma Definition: Search for: Glossary - word Glossary - def Textbooks Protocols Images Tools Forum PubMed Links Press Releases Biology Glossary search by EverythingBio.com A cancer of the human retina. Predisposition to retinoblastoma is inherited as an autosomal dominant. A childhood cancer of retinoblast cells caused by the inactivation of an anti-oncogene. A B C D ... Home Website created and maintained by: Mark Lefers and the Holmgren Lab last updated: July 26, 2004

76. Interactive Fly, Drosophila Drosophila retinoblastomafamily protein (Rbf) was isolated by virtue of its ability Mutants of Rbf (a retinoblastoma protein homolog) fail to limit DNA http://www.sdbonline.org/fly/newgene/retnbls1.htm

Retinoblastoma-family protein Biological Overview Evolutionary Homologs Regulation Developmental Biology ... References Gene name - Retinoblastoma-family protein Synonyms - RBF Cytological map position - Function - transcription factor Keywords - cell cycle Symbol - Rbf FlyBase ID: Genetic map position - Classification - Retinoblastoma family Cellular location - presumably nuclear NCBI and GadFly links: Precomputed BLAST LocusLink GadFly Fly GRID Rbf genetic and physical interactions BIOLOGICAL OVERVIEW In mammalian cells, the Retinoblastoma protein (RB) acts as a critical switch, regulating entry into the DNA synthetic phase (S phase) of the cell cycle . RB interacts with and negatively regulates members of a family of factors called E2Fs, which serve to activate transcription of genes required for entry into S phase. Phosphorylation of RB by cyclin/cyclin dependent kinases, the protein dimers that regulate the cell cycle, target RB for destruction, releasing E2F, thereby allowing E2F to carry out its function as a transcriptional activator. In mammals, RB is represented by a family of three closely related proteins: RB itself, p107 and p130. Drosophila Retinoblastoma-family protein (Rbf) was isolated by virtue of its ability to interact with E2F complexes containing Drosophila

77. Retinoblastoma In Children - Keep Kids Healthy Conditions retinoblastoma is the most common tumor of the eye in children and is often found when checking an infant s red reflex and finding leukocoria (white http://www.keepkidshealthy.com/welcome/conditions/retinoblastoma.html

Bookstore Site Map Contact Us Help ... What's New? Search this site: Advanced Search Main Menu Useful Tools Index of Topics Pediatric Problems Parenting Tips ... Web Links Online Resources What's New Reviews Growth Charts Online Forums ... Height Calculator Newsletters: Subscribe to get free news, tips and updates. Recommend Us tell a friend about us or email this page to a friend Main Diseases and Conditions Retinoblastoma Although uncommon and occurring in only 1 in 18,000 to 20,000 live births, retinoblastoma is the most common tumor of the eye and usually occurs in the first few years of a child's life and most cases (90%) occur before age 5. Although not as common, older children can get retinoblastoma too, and they usually complain of eye pain or decreased vision in the affected eye. Retinoblastoma is responsible for causing 5% of the cases of childhood blindness. There is a tumor suppressor gene on the long arm of chromosome 13 and it is the retinoblastoma gene. The presence of this gene helps to suppress retinoblastoma from forming by forming a protein that is though to help regulate cell growth. If both copies of the gene are mutated or absent, then retinoblastoma can form. In familial or genetic forms of retinoblastoma, one copy of the defective gene is usually inherited and the other one becomes mutated. In non hereditary cases of retinoblastoma, both genes become mutated. Retinoblastoma is commonly found during routine screening in high risk children, specifically newborns that have other family members with retinoblastoma. Other infants are diagnosed when they are found to not have a red reflex (or red eye) during a routine well baby checkup or if the child's eyes appear different in a photograph. Instead, they have leukocoria (cat's eye reflex) or a white pupillary reflex. Later symptoms can include redness or inflammation of the eye, strabismus or abnormalities of the pupil.

78. Childhood Eye Cancer Trust (CHECT) | Fighting Retinoblastoma Report on Living with retinoblastoma the psychological and social consequences. Welcome to the Childhood Eye Cancer Trust. This website is for anyone who http://www.chect.org.uk/

Skip navigation Home The Childhood Eye Cancer Trust (CHECT) is a UK wide charity for families and individuals affected by retinoblastoma. We offer support and information, fund research and raise public awareness of this rare cancer. About Retinoblastoma What is Retinoblastoma Diagnosis and treatment Long-term consequences ... International What's New Awareness week: 11th - 17th September Help us spread awareness of Rb and CHECT's work! £1 for life Our handy new CHECT coin is now available! Annual Family Day Saturday 17th September at Birmingham Children's Hospital. Details and booking form. Welcome to the Childhood Eye Cancer Trust. This website is for anyone who wishes to know more about the charity, about retinoblastoma, or how to play a part in our vision. Find out more about us Donate now The work of The Childhood Eye Cancer Trust is entirely funded by donations, so your support is vital to us. Join now Become a member of the Childhood Eye Cancer Trust and help us fight cancer. Annual Family Day Saturday 17th September at Birmingham Children's Hospital. Click here for details! Childhood Eye Cancer Trust :: The Royal London Hospital :: Whitechapel Road :: London E1 1BB Registered Charity No. 327493

79. Retinoblastoma - Genetics Home Reference Where can I find additional information about retinoblastoma? What if I still have specific questions about retinoblastoma? http://ghr.nlm.nih.gov/condition=retinoblastoma

Home What's New Browse Handbook ... Search Retinoblastoma Related Gene(s) Quick links to this topic NIH Publications National Institutes of Health MedlinePlus Health Information Genes and Disease Genetic disorder summaries Educational resources Information pages Patient support For patients and families Gene Reviews Clinical summary Gene Tests DNA test labs ClinicalTrials.gov Research studies PubMed Recent literature OMIM Genetic disorder catalog References Retinoblastoma On this page: What is retinoblastoma? How common is retinoblastoma? ... help with understanding retinoblastoma? What is retinoblastoma? Retinoblastoma is a rare type of eye cancer that develops in the retina, which is the part of the eye that detects light and color. Although this disorder can occur at any age, it usually develops in young children. Most cases of retinoblastoma occur in only one eye, but both eyes can be affected. The most common sign of this disorder is a visible whiteness in the normally black pupil (the part of the eye that lets in light). This unusual whiteness is particularly noticeable in photographs taken with a flash. This sign is called "cat's eye reflex" or leukocoria. Other signs and symptoms of retinoblastoma include crossed eyes or eyes that do not point in the same direction (strabismus); persistent eye pain, redness, or irritation; and blindness or poor vision in the affected eye. People with the hereditary form of retinoblastoma may also develop a tumor in the brain called pinealoma. Pinealoma develops in the pineal gland, which is located at the base of the skull. The presence of retinoblastoma and pinealoma together is called trilateral retinoblastoma. People with hereditary retinoblastoma also have an increased risk of developing other types of cancer, such as bone cancer (osteosarcoma), later in life.

80. Oncology - Retinoblastoma In inherited retinoblastoma (40 percent of the cases), the first mutation is However, retinoblastoma has been linked to ovarian, breast, lung, prostate, http://www.schneiderchildrenshospital.org/peds_html_fixed/peds/oncology/retino.h

Oncology Retinoblastoma What is retinoblastoma? Retinoblastoma is a rare cancer of the retina. The retina is the innermost layer of the eye, located at the back of the eye, that receives light and images necessary for vision. What causes retinoblastoma? Retinoblastoma occurs due to mutations in a tumor suppressor gene (called RB1) located on chromosome #13. Two mutations (or gene changes) are necessary to "knock-out" this gene, and cause uncontrolled cell growth. In inherited retinoblastoma (40 percent of the cases), the first mutation is inherited from a parent, while the second occurs during the development of the retina. In sporadic retinoblastoma (60 percent of the cases), both mutations occur during development of the retina. Sporadic means "occurs by chance." Alterations in the RB1 gene have also been found in other tumors, including osteosarcoma and breast cancer. Most children with inherited retinoblastoma generally have tumors involving both eyes. (In fact, all cases involving both eyes should be considered hereditary). The RB1 gene is an autosomal dominant gene, which means that both males and females are equally affected, and there is a 50/50 chance, with each pregnancy, for a parent to transmit the gene to a child. When a child inherits the gene, there is a 75 to 90 percent chance for the second mutation to occur, resulting in retinoblastoma. This means that some children who inherit the mutation may never get the second mutation, and may, therefore, never develop retinoblastoma. (They can still transmit the gene to their offspring, however, so that their children could develop the disease.)

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