21. Oncology - Retinoblastoma Describes key information about retinoblastoma in note format. http://www.mmhs.com/clinical/peds/english/oncology/retino.htm

Site Map English - Adult English - Pediatric Spanish - Adult ... Online Resources 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.)

22. Retinoblastoma Covers cellular classification, staging, treatment options, and types of retinoblastoma, with references for each topic. http://www.uoc.muni.cz/guidelines/1deti/RETINOBL.htm

Retinoblastoma Table of Contents GENERAL INFORMATION CELLULAR CLASSIFICATION STAGE INFORMATION Intraocular Extraocular Reese-Ellsworth classification for intraocular tumors St. Jude Children's Research Hospital clinical staging system TREATMENT OPTION OVERVIEW INTRAOCULAR RETINOBLASTOM A Unilateral disease Bilateral disease EXTRAOCULAR RETINOBLASTOMA RECURRENT RETINOBLASTOMA GENERAL INFORMATION This treatment information summary on retinoblastoma is an overview of prognosis, diagnosis, classification, and treatment. The National Cancer Institute created the PDQ database to increase the availability of new treatment information and its use in treating patients. Information and references from the most recent published literature are included after review by pediatric oncology specialists. Cancer in children is rare. A team approach, incorporating the skills of the local physician, radiation therapists, pediatric medical oncologists/hematologists, rehabilitation specialists, and social workers, is imperative to ensure that patients receive the best treatment. In order for advances to be made in treating these patients, therapy is best delivered in the context of a clinical trial at a major medical center that has expertise in treating children. Only through entry of all eligible children into appropriate, well-designed clinical trials will progress be made against these diseases. Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[1]

23. Retinoblastoma Academic review article about developments in the diagnosis and treatment of retinoblastoma. http://www.moffitt.usf.edu/pubs/ccj/v5n4/article2.html

Retinoblastoma Curtis E. Margo, MD, MPH, Lynn E. Harman, MD, and Zuber D. Mulla, MSPH Although treatment outcomes for retinoblastoma have improved, patients with a germline gene mutation carry a high risk for second cancers. Background: Retinoblastoma is the most common intraocular malignancy of infants and children. With early diagnosis and treatment, survival is greater than 90%; however, patients with a germline retinoblastoma mutation have a substantial risk of having a second high-grade malignancy. Methods: The recent developments in the diagnosis and treatment of retinoblastoma are reviewed. Results: Identification of the retinoblastoma germline mutation is now possible with the discovery of the retinoblastoma gene. Patients with the germline mutation have a 51% cumulative risk over 50 years of developing a second malignancy. Several pilot studies using primary chemotherapy for retinoblastoma have shown promising results. Conclusions: Risk assessment and genetic counseling have become more precise with the development of laboratory methods to identify the retinoblastoma gene. The development of primary chemotherapy regimens to reduce the size of retinoblastoma tumors may decrease the need for radiation therapy and thereby reduce the risk of radiation-related malignancies in patients with the germline mutation. Introduction Retinoblastoma is the most common intraocular malignancy of infancy and childhood. Prior to this century, retinoblastoma was a uniformly fatal disease.

24. Understanding Retinoblastoma Childhood Eye Cancer Trust link to home page. Understanding retinoblastoma. What is retinoblastoma? How common is retinoblastoma? http://www.rbsociety.org.uk/about_rb/understanding_rb.htm

Understanding Retinoblastoma What is retinoblastoma? Retinoblastoma is a malignant tumour or cancer which develops in the cells of the retina, the light sensitive lining of the eye (see diagram). More than nine out of ten children with retinoblastoma can be cured and it has one of the best cure rates of all children's cancers. In about two thirds of children only one eye is affected but in one third, tumours develop in both eyes. When only one eye is affected this is known as unilateral retinoblastoma and when both eyes are affected, it is called bilateral retinoblastoma. In very young children, who have only one eye affected at diagnosis, it is possible for a tumour to develop in the second eye several weeks or even months after the diagnosis of retinoblastoma in the first eye. The number of tumours found in the eye also varies; sometimes only one tumour develops at the back of the eye but not infrequently there are several tumours, and these may require more than one type of treatment. How common is retinoblastoma?

25. Transpupillary Thermotherapy (TTT) And Transscleral Thermotherapy (TSTT) Research information on laser treatments for intraocular tumors, such as choroidal melanoma, retinoblastoma, and hemangioma. http://members.ams.chello.nl/rem/tstt/

Transpupillairy Thermotherapy and Transscleral Thermotherapy for treatment of intraocular tumors Home TTT TTT basics TSTT ... Contact us Research information on Transpupillary Thermotherapy, TTT, and Transscleral Thermotherapy, TSTT, for treatment of intra-ocular tumors: choroidal melanoma, hemangioma, retinoblastoma. Purpose To inform other investigators interested in medical, clinical and technical aspects of laser heat treatments, such as hyperthermia and thermotherapy, in ocular oncology. TTT and TSTT TTT is a new laser heat-treatment modality for patients with a choroidal melanoma. TSTT is an experimental laser treatment for tumors in the eye. TTT and TSTT are one of many applications of medical lasers in the field of ophthalmology. New articles TTT Journee-de Korver JG, Keunen JE. Thermotherapy in the management of choroidal melanoma. Prog Retin Eye Res. 2002 May;21(3):303-17. New articles TSTT Rem AI, Oosterhuis JA, Korver JG, van den Berg TJ.

26. ACS :: Detailed Guide: Retinoblastoma (An Eye Cancer) Comprehensive overview of risks, causes, prevention, detection, diagnosis, staging, and treatment of retinoblastoma. http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=37

27. Cancer.gov - URL Changed What You Need To Know About Cancer An Overview. An overview of cancer detection, symptoms, diagnosis, and treatment. NIH Publication No. 00-1566. Questions and Answers About Care for Children and http://www.nci.nih.gov/cancer_information/cancer_type/retinoblastoma

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28. DJO Digital Journal Of Ophthalmology Patient Information. Submit Patient Info. Register with DJO to receive personalized updates. If you're already a. member, please sign in. retinoblastoma. Printer Friendly. Peter K. Kaiser, MD. Ingrid http://www.djo.harvard.edu/meei/PI/RB/RB.html

29. Retinoblastoma.Net: What Is Retinoblastoma? What Is retinoblastoma? retinoblastoma is a relatively uncommon tumor of childhood that accounts for about 3% of the cancers in children under the age of 15. http://www.retinoblastoma.net/WhatIsRB/whatisrb.aspx

RBI Home Parents, Family, What We ... Medical Terms What Is Retinoblastoma? Retinoblastoma (reh-tin-oh-blast-oma) is a childhood cancer arising from immature retinal cells in one or both eyes and can strike from the time a child is in the womb up to 5 years of age. This cancer is curable if caught early enough. However, 87% of the children stricken with this disease worldwide die, mostly in developing countries. In developed countries, 97% of those who do live have moderate to severe visual impairment. Retinoblastoma is a relatively uncommon tumor of childhood that accounts for about 3% of the cancers in children under the age of 15. The tumors originate in the retina, the light sensitive layer of the eye, which enables the eye to see. When the tumors are present in one eye, it is referred to as unilateral retinoblastoma, and when it occurs in both eyes it is referred to as bilateral retinoblastoma. 60% of the cases involve only one eye (unilateral); the rest affect both eyes (bilateral). 90% of retinoblastoma patients have no family history of the disease and only 10% of newly diagnosed patients have other family members with retinoblastoma. Although it is rare, retinoblastoma can spread or metastasize outside of the eye to the brain, the central nervous system (brain and spinal cord), and the bones. In these cases, chemotherapy is prescribed by a pediatric oncologist and is administered through the peripheral blood vessels or into the brain for months to years after initial diagnosis of metastatic disease.

30. The National Retinoblastoma Research And Support Foundation Network of local groups give support to families with this condition. http://www.djo.harvard.edu/meei/PI/RB/NRRSF.html

31. Retinoblastoma Treatment,Symptoms Of Retinoblastoma,Eye Cancer Retinoblastoma,Re A set of questions and answers about retinoblastoma. http://www.visitech.org/retinoblastoma.html

Seek An Opinion Services Infrastructure Quality Policy ... Know Us Retinoblastoma What is retinoblastoma? Retinoblastoma is a cancer of the eye in children. It originates from the retina, the light sensitive layer, in eye. It is the commonest eye tumor of the eye in childhood. . This tumor may involve one (75% of cases) or both (25% of cases) eyes in a child. Untreated, retinoblastoma is almost always fatal, hence the importance of early diagnosis and treatment Is it hereditary? Yes in some cases it may be hereditary. Majority of cases (90%) has no family history of such disease; while a small percentage (10%) of newly diagnosed cases have other family members with retinoblastoma. Of all the cases of retinoblastoma, in 60% of cases it does not get transmitted to the next generation, while in 40% of cases it may get transmitted in the next generation. Therefore it is important to have a genetic counseling before having a baby to determine the risk of another child getting the same disease. Also, the siblings and children of the patients with retinoblastoma should be examined periodically in their childhood to detect any possible tumor early. What are the symptoms of retinoblastoma?

32. THE MERCK MANUAL, Sec. 19, Ch. 266, Neoplasms Information on this type of intraocular tumor from an online textbook. http://www.merck.com/pubs/mmanual/section19/chapter266/266d.htm

This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 19. Pediatrics Chapter 266. Neoplasms Topics [General] Wilms' Tumor Neuroblastoma Retinoblastoma Retinoblastoma A malignant tumor that arises from the immature retina. Retinoblastoma occurs in 1/15,000 to 1/30,000 live births and represents about 2% of childhood malignancies. The disease may be inherited or result from a new germinal mutation. About 10% of patients have a family history of retinoblastoma and another 20 to 30% have bilateral disease; all of these (ie, 30 to 40% of the patients) may pass the trait to their children in an autosomal dominant fashion. These patients appear to have a constitutive genetic abnormality, which in at least 25% appears to be a deletion involving chromosome 13q14 (smaller undetectable abnormalities may be present in all these patients). A mutation in the other chromosome 13 (second hit) is thought to result in the tumor. Most of the remaining 60% of patients, with unilateral disease and no family history of retinoblastoma, have nonheritable disease. However, about 5% of these patients may also carry the gene for retinoblastoma with the risk of passing the trait to their children. Diagnosis Diagnosis is usually made by age 3 to 4 yr when a white reflex from the pupil (cat's-eye pupil) or strabismus is investigated. Both fundi must be closely examined by indirect ophthalmoscopy with the pupils widely dilated and the child under general anesthesia. The tumors appear as single or multiple gray-white elevations in the retina; tumor seeds may be visible in the vitreous. In almost all tumors, calcification can be detected by CT.

33. RETINOBLASTOMA - Meaning And Definition Of The Word Definition of retinoblastoma, with links to each word in the definition for further explanations. http://www.hyperdictionary.com/dictionary/retinoblastoma

English Dictionary Computer Dictionary Thesaurus Dream Dictionary ... Medical Dictionary Search Dictionary: RETINOBLASTOMA: Dictionary Entry and Meaning Pronunciation: r`etun`owbl`ast'owmu WordNet Dictionary Definition: [n] malignant ocular tumor of ... cells See Also: malignant neoplasm malignant tumor metastatic tumor Medical Dictionary Definition: An eye cancer that most often occurs in children younger than 5 years. It occurs in hereditary and nonhereditary (sporadic) forms. Biology Dictionary Definition: Retinoblastoma is the most common malignant intraocular neoplasm of infants, about half of all children with retinoblastoma may be inherited as an autosomal dominant trait, in which case, the cancer afflicts both eyes. Glossary Definition: a cancer of the retina of the eye in children, inherited as a dominant. HOME ABOUT HYPERDICTIONARY

34. Retinoblastoma Básico Translate this page A web site for patients with eye cancer of within around and behind the eyes including but not limited to choroidal melanoma,care,retinoblastoma,eye,lid,tumors http://www.eyecancer.com/Spanish/conditionsS/RetinalTumorsS/retinoS.html

El editor: Dr. Paul Finger El ojo Glosario Preguntas Frecuentes ... Enlaces Tratamentos: Radioterapia Innovaciones Estudios Fundación "eyecare" ... Tienda de ECN Retinoblastoma pupila blanca leucocoria Tratamiento Un Caso de Retinoblastoma - Medscape Interactivo Link here for multiple clinical photos (Click the Reference to Read the Abstract): 1. Quimioterapia para Retinoblastoma. A Current Topic Finger PT, Czechonska G, Demirci H, Rausen A. Drugs 1999;58(6):983-996. SUBIR AL PRINCIPIO Por favor informe de cualquier problema a: pfinger@eyecancer.com Paul T. Finger,MD, FACS 1998-2003 SE RESERVAN TODOS LOS DERECHOS 115 East 61st Street New York City, New York 10021

35. Retinoblastoma Information in note form for clinicians. http://www.fpnotebook.com/EYE45.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 General Medicine Geriatric Medicine Gynecology Hematology and Oncology HIV Infectious Disease Jokes Laboratory Neonatology Nephrology Neurology Obstetrics Ophthalmology Orthopedics Otolaryngology Pediatrics Pharmacology 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%

36. Retinoblastoma Genes and Disease PDF Document Diseases of the Eye retinoblastoma retinoblastoma occurs in early childhood and affects about 1 child in 20,000. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?call=bv.View..ShowSection&rid=gnd.sect

37. National Cancer Institute - Retinoblastoma Home Page Information and resources for this condition. http://www.cancer.gov/cancerinfo/types/retinoblastoma

var bSearchBoxBool=false; window.onload+="MM_preloadImages('/images/nav_home_over.gif','/images/nav_cancer_topics_over.gif','/images/nav_clinical_trials_over.gif','/images/nav_cancer_statistics_over.gif','/images/nav_research_funding_over.gif','/images/nav_news_over.gif','/images/nav_about_over.gif');" Quick Links Dictionary Funding Opportunities NCI Publications NCI Calendar NCI Highlights Some Men with Low PSAs Have Prostate Cancer Energy Balance Trans-HHS Cancer Health Disparities Report Past Highlights Retinoblastoma Related Pages What You Need To Know About Cancer - An Overview An overview of cancer detection, symptoms, diagnosis, and treatment. NIH Publication No. A fact sheet about children's cancer centers and health care approaches, including clinical trials for children with cancer. Young People with Cancer: A Handbook for Parents Discusses the most common types of childhood cancer, treatments and side effects, and issues that may arise when a child is diagnosed with cancer. Offers medical information and practical tips gathered from parents. Includes a section on talking to children about cancer. Full description of the NCI PDQ database.

38. The Retinoblastoma Protein- A Master Regulator Of Cell Cycle, Differentiation An Comprehensive scientific overview about the retinoblastoma protein. http://hucellbiol.mdc-berlin.de/review0_text.htm

The retinoblastoma protein- a master regulator of cell cycle, differentiation and apoptosis Susanne Herwig and Michael Strauss Danish Cancer Society, Division for Cancer Biology, Department of Cell Cycle and Cancer, Strandboulevarden 49, DK-2100 Copenhagen, Denmark correspondence Abbreviations pRb, retinoblastoma protein; , retinoblastoma gene; SV40 Tag, Simian virus 40 large T antigen; HPV, human papilloma virus; Pol, RNA polymerase; H folate reductase, dihydrofolate reductase; TK, thymidine kinase; RbK, retinoblastoma kinase; Cdk, cyclin-dependent kinase; PCNA, proliferating cell nuclear antigen; TGF-b1, transforming growth factor b1; IFN-g, interferon-g; Cki, cyclin-dependent kinase inhibitor; TS, thymidylate synthase. Key words : retinoblastoma, cell cycle, restriction point, differentiation, apoptosis Acknowledgements We thank Michael Gotthardt for artwork. Summary The retinoblastoma susceptibility gene is a tumour suppressor and its product, pRb, is known as a repressor of progression towards S phase for 10 years. Its major activity was supposed to be sequestration or inactivation of the transcription factor E2F which is required for activation of S phase genes. However, within recent years growing evidence has been accumulating for a more general function of pRb at both, the transcriptional level as well as the cellular level. pRb not only regulates the activity of certain protein-encoding genes but also the activity of pol I and pol III transcription. This protein appears to be the major player in a regulatory circuit in the late G

39. CancerBACUP : Retinoblastoma This section is about retinoblastoma in children. It describes what they are, symptoms, diagnosis and treatment. Search. retinoblastoma. http://www.cancerbacup.org.uk/Cancertype/Childrenscancers/Typesofchildrenscancer

@import "/stylesheet-site.css"; Search Retinoblastoma This section gives information about retinoblastoma in children. It is helpful to read it alongside CancerBACUP's general information children's cancers, which contains more information about cancers in children, their diagnosis and treatment and the support services available. There are about 40 new cases of retinoblastoma diagnosed each year in the UK. What is retinoblastoma? What causes retinoblastoma? What are the signs and symptoms? How it is diagnosed ... Follow-up Diagram of the eye (d) back to top What is retinoblastoma? Retinoblastoma is a tumour that occurs in the light sensitive lining of the eye known as the retina. It can occur in two forms, an inherited form where there are often tumours in both eyes and a non-inherited form where there is usually only a tumour in one eye. back to top What causes retinoblastoma? In the inherited tumours (which are about two cases in every five), it is caused by a genetic abnormality. This means that an abnormal gene, passed from a parent to the child, allows the tumour to develop. Patients with this gene, known as the Rb gene, also have a slightly increased risk of developing other tumours outside the eye. Genetic counselling and support is available for all families with a diagnosis of retinoblastoma. The cause of the non-inherited tumours is unknown. back to top What are the signs and symptoms?

40. National Cancer Institute - Retinoblastoma Home Page retinoblastoma Home Page. What therapy. retinoblastoma Treatment patients health professionals . Cryosurgery in Cancer Treatment Q A. http://www.nci.nih.gov/cancerinfo/types/retinoblastoma/

var bSearchBoxBool=false; window.onload+="MM_preloadImages('/images/nav_home_over.gif','/images/nav_cancer_topics_over.gif','/images/nav_clinical_trials_over.gif','/images/nav_cancer_statistics_over.gif','/images/nav_research_funding_over.gif','/images/nav_news_over.gif','/images/nav_about_over.gif');" Quick Links Dictionary Funding Opportunities NCI Publications NCI Calendar NCI Highlights Some Men with Low PSAs Have Prostate Cancer Energy Balance Trans-HHS Cancer Health Disparities Report Past Highlights Retinoblastoma Related Pages What You Need To Know About Cancer - An Overview An overview of cancer detection, symptoms, diagnosis, and treatment. NIH Publication No. A fact sheet about children's cancer centers and health care approaches, including clinical trials for children with cancer. Young People with Cancer: A Handbook for Parents Discusses the most common types of childhood cancer, treatments and side effects, and issues that may arise when a child is diagnosed with cancer. Offers medical information and practical tips gathered from parents. Includes a section on talking to children about cancer. Full description of the NCI PDQ database.

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