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Drugs 2007Survival rates for retinoblastoma patients have increased dramatically over the last century, with documented 5-year survival figures reaching 87-99% in developed... (Review)
Review
Survival rates for retinoblastoma patients have increased dramatically over the last century, with documented 5-year survival figures reaching 87-99% in developed countries. During the last decade, there has been a dramatic paradigm shift in the treatment approach for intraocular retinoblastoma, emphasising chemoreduction protocols and minimising the use of external beam radiation. The recognition of the increased risk for second non-ocular cancers with external beam radiation contributed to the growing emergence of chemotherapy. Although chemoreduction protocols vary slightly between institutions, many centres are currently treating intraocular retinoblastoma with carboplatin, vincristine and etoposide as a three-drug regimen given in two to six cycles. Clinical studies have demonstrated that systemic chemotherapy must be combined with other modalities, such as laser treatment and cryotherapy, for adequate tumour control and many eyes with advanced intraocular disease require salvage therapy with radiation or enucleation. Therefore, modern centres treating retinoblastoma continue to manage patients with a variety of modalities, individualising the therapy according to the patient's presentation and clinical course.
Topics: Animals; Antineoplastic Agents; Brachytherapy; Combined Modality Therapy; Eye Enucleation; Humans; Immunotherapy; Retinal Neoplasms; Retinoblastoma
PubMed: 17927283
DOI: 10.2165/00003495-200767150-00005 -
Ophthalmology Apr 1987Anterior chamber retinoblastoma is a rare clinical entity. The authors have reviewed the records of 1500 patients with retinoblastoma to determine the incidence and...
Anterior chamber retinoblastoma is a rare clinical entity. The authors have reviewed the records of 1500 patients with retinoblastoma to determine the incidence and prognostic ramifications. Of 30 patients with anterior chamber involvement, 15 were noted on initial examination and 15 during observation after therapy. Despite aggressive multimodality treatment, total response was never achieved and all eyes were eventually lost. Results of pathologic examination showed ciliary body invasion, which was held accountable for the poor response to therapy. Anterior chamber retinoblastoma is a poor prognostic sign which cannot be controlled effectively with current techniques and should be considered an indication for enucleation without delay.
Topics: Child; Child, Preschool; Eye Neoplasms; Female; Follow-Up Studies; Humans; Infant; Male; Prognosis; Retinoblastoma
PubMed: 3587918
DOI: 10.1016/s0161-6420(87)33437-2 -
Pediatric Hematology and Oncology Apr 2020
Clinical Trial
Topics: Adolescent; Botswana; Child; Child, Preschool; Female; Humans; Infant; Institutional Practice; Male; Physicians; Retinoblastoma
PubMed: 31858896
DOI: 10.1080/08880018.2019.1704952 -
Archives of Ophthalmology (Chicago,... Jul 1999To evaluate the results of thermotherapy for retinoblastoma.
OBJECTIVE
To evaluate the results of thermotherapy for retinoblastoma.
DESIGN
Prospective, nonrandomized analysis of the treatment method.
PARTICIPANTS
A total of 188 retinoblastomas in 80 eyes of 58 patients who were treated with thermotherapy.
MAIN OUTCOME MEASURES
Tumor response and ocular adverse effects.
RESULTS
Of 188 retinoblastomas treated with thermotherapy, mean tumor base was 3.0 mm and tumor thickness was 2.0 mm. Complete tumor regression was achieved in 161 tumors (85.6%), and 27 tumors (14.4%) developed recurrence. Using univariate analysis, the predictors of local tumor recurrence were male sex (P = .005), no color change ("no visible take") in tumor after treatment (P = .01), increasing number of treatment sessions (P = .002), and previous use of chemoreduction (P = .02). By multivariate analysis, the most important predictors of local tumor recurrence were male sex (P = .01) and previous use of chemoreduction (P = .03), the latter likely reflecting the fact that these tumors were initially larger with more ominous findings, and required chemoreduction therapy to reduce them to a size amenable to focal treatment with thermotherapy. When evaluating thermotherapy variables as a function of tumor size, it was apparent that larger tumors (> or =3.0-mm base) required greater energy and time than did smaller tumors (<3.0-mm base). Comparison of treatment variables for larger vs smaller tumors was as follows: number of treatment sessions, 3.3 vs 2.3; spot size, 1.7 vs 1.3 mm; power, 540 vs 370 mW; treatment duration, 49 vs 14 minutes; and coupling of thermotherapy with chemotherapy, 79% vs 48% of cases (P < or =.001 for each variable). Complications of thermotherapy in the 80 eyes included focal iris atrophy in 29 eyes (36%), peripheral focal lens opacity in 19 eyes (24%), retinal traction in 4 eyes (5%), retinal vascular obstruction in 2 eyes (2%), and transient localized serous retinal detachment in 2 eyes (2%). There were no cases of corneal scarring, central lens opacity, iris or retinal neovascularization, or rhegmatogenous retinal detachment. All eyes with focal lens opacity demonstrated adjacent focal iris atrophy. By multivariate analysis, the predictors of thermotherapy-induced focal iris atrophy were increasing number of treatment sessions (P = .001) and increasing tumor base (P = .02).
CONCLUSIONS
Thermotherapy is used for relatively small retinoblastomas without associated vitreous or subretinal seeds. This treatment provides satisfactory control for selected retinoblastomas, with 86% of tumors demonstrating lasting regression. Tumors that measure 3.0 mm or larger in base at the time of thermotherapy require more intense treatment than smaller tumors and are at greatest risk for ocular complications such as focal iris atrophy and focal paraxial lens opacity.
Topics: Chemotherapy, Adjuvant; Child; Child, Preschool; Female; Fluorescein Angiography; Fundus Oculi; Humans; Hyperthermia, Induced; Infant; Male; Neoplasm Recurrence, Local; Prospective Studies; Retinal Neoplasms; Retinoblastoma; Treatment Outcome
PubMed: 10408452
DOI: 10.1001/archopht.117.7.885 -
Soins. Gynecologie, Obstetrique,... May 1991
Topics: Diagnosis, Differential; Eye Enucleation; Eye Neoplasms; Humans; Infant; Prognosis; Retinoblastoma
PubMed: 2047988
DOI: No ID Found -
Revista Chilena de Pediatria 1979
Topics: Child, Preschool; Eye Neoplasms; Female; Humans; Infant; Infant, Newborn; Male; Retinoblastoma
PubMed: 545450
DOI: 10.4067/s0370-41061979000300005 -
Ceskoslovenska Oftalmologie Mar 1973
Topics: Antineoplastic Agents; Child, Preschool; Eye Neoplasms; Female; Humans; Infant; Male; Retinoblastoma
PubMed: 4694130
DOI: No ID Found -
Archivos de Neurobiologia 1970
Topics: Diagnosis, Differential; Female; Humans; Male; Retinoblastoma
PubMed: 5424883
DOI: No ID Found -
Asian Pacific Journal of Cancer... 2004The objective was to assess epidemiologic aspects of retinoblastoma development in Karachi, Pakistan. Incident cases, diagnosed clinically or microscopically and... (Comparative Study)
Comparative Study
The objective was to assess epidemiologic aspects of retinoblastoma development in Karachi, Pakistan. Incident cases, diagnosed clinically or microscopically and registered at Karachi Cancer Registry (KCR) during 1(st)January 1998 to 31(st) December 2002 were reabstracted, rechecked and reanalyzed for this purpose. One hundred and one cases of retinoblastoma were reported to KCR over the 5 years (1998-2002). Fifty-seven were residents of Karachi, 34 (59.6%) males and 23 (40.4%) females. The gender ratio (M:F) was 1.5. The mean age at diagnosis was 3.96 years (95% CI 2.92; 4.99) and 3.85 years (95% CI 2.72; 4.98) in males and females respectively. The annual crude incidence of retinoblastomas in Karachi was 4.0/100,000 and 2.4/100,000 in children under the age of 5 and 10 years respectively, the corresponding age standardized rates being 5.3/100,000 and 4.8/100,000. The age groups at risk of developing retinoblastoma, associated morbidity and possibility of almost 100% 5-year survival with available treatments, calls for ophthalmologic screening of all infants below 1 year, and high-risk children until the age of 7 years. In order to detect retinoblastoma, as early as possible, health education for parents and health providers, and improved training of ophthalmologists is essential. Genetic testing for siblings and children of retinoblastoma cases and identification of high-risk children would be helpful, but lacks financial feasibility in developing countries at present. Future health care planning should focus on capacity building for neonatal ophthalmologic screening, handling of parents'and children'emotional reactions and opportunities for education, occupational training and cosmetic rehabilitation for surviving retinoblastoma patients.
Topics: Adolescent; Adult; Age Distribution; Aged; Child; Child, Preschool; Cohort Studies; Developing Countries; Female; Humans; Incidence; Infant; Male; Middle Aged; Pakistan; Retinal Neoplasms; Retinoblastoma; Retrospective Studies; Risk Assessment; Sex Distribution; Survival Analysis; Urban Population
PubMed: 15244518
DOI: No ID Found -
Asian Pacific Journal of Cancer... 2006Molecular genetic diagnostics for retinoblastoma are prerequisite for accurate risk prediction and effective management. Developing a retinoblastoma diagnostic model to...
PURPOSE
Molecular genetic diagnostics for retinoblastoma are prerequisite for accurate risk prediction and effective management. Developing a retinoblastoma diagnostic model to establish a flow for laboratory tests is thus a necessity for tertiary ophthalmic institutions. An efficient diagnostic model could reduce the overall health care costs, redirect the resources to the high risk group and also avoid unnecessary worry for families. To the best of our knowledge there has hitherto been no comprehensive diagnostic model for retinoblastoma implemented in any institution in India.
METHODS AND DISCUSSION
The diagnostic model demonstrates the logical and practical flow of various genetics tests like karyotyping, loss of heterozygosity analysis, molecular deletion, linkage analysis (familial cases), mutation screening of -CGA exons first and then non-CGA exons, methylation screening of RB1 and essential promoter regions screening in a laboratory.
CONCLUSIONS
The diagnostic model proposed offers acomprehensive methodology to identify the causative two-hits for retinoblastomas that could be used while genetic counseling families. This model is applicable in tertiary hospitals in India and neighboring countries, which have the highest incidence of retinoblastoma and fertility rates in the world. We suggest that this diagnostic model could also be applied with modification for other cancers.
Topics: Child; Child, Preschool; Cost-Benefit Analysis; Diagnostic Techniques, Ophthalmological; Genetic Testing; Humans; India; Models, Biological; Retinal Neoplasms; Retinoblastoma; Retinoblastoma Protein
PubMed: 17059352
DOI: No ID Found