Purpose: To determine the efficacy of primary intravenous chemotherapy (IVC) plus secondary intraarterial chemotherapy (IAC) for patients with advanced retinoblastoma.
Methods: Retrospective, nonrandomized interventional case series of 14 patients with retinoblastoma managed with primary systemic IVC (vincristine, etoposide, and carboplatin for 6 cycles) followed by secondary IAC (melphalan for 1-6 cycles).
Results: Fourteen patients with advanced retinoblastoma classified by the International Classification of Retinoblastoma as Group D (n = 6, 43%) or Group E (n = 8, 57%) were treated with IVC as primary treatment and subsequent secondary IAC as rescue or consolidation therapy. The IAC was given for recurrent retinoblastoma and/or subretinal/vitreous seeds in 13 eyes (93%) and for persistent viable retinoblastoma in 1 eye (7%). Enucleation was the alternative option. The mean interval between IVC completion and IAC start was 40 weeks (median, 11 weeks; range, 2-170 weeks) and the mean number of IAC cycles was 3 (median, 3; range, 1-6). After primary IVC plus secondary IAC, globe salvage was achieved in 8 patients (57%) at mean 2-year follow-up. There was no evidence of retinoblastoma metastasis or death and no sign of second cancer or life-threatening complication.
Conclusion: For advanced retinoblastoma (Groups D and E) in which enucleation is the alternative option, primary systemic IVC followed by secondary focal IAC provides globe salvage in 57% of the eyes and with no metastatic event.
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http://dx.doi.org/10.1097/IAE.0b013e318295f783 | DOI Listing |
Ethiop J Health Sci
October 2024
Radiologists, Addis Ababa University, College of Health Sciences, Medical Faculty, Department of Radiology, Addis Ababa, Ethiopia.
Background: Retinoblastoma is the most prevalent intraocular retinal malignancy in children worldwide. Accurate staging is critical for treatment planning and relies heavily on radiologic imaging and clinical findings. This study aims to evaluate imaging patterns and staging of retinoblastoma in children at Tikur Anbessa Specialized Hospital (TASH).
View Article and Find Full Text PDFJCI Insight
December 2024
Department of Ophthalmology and Roger and Karalis Johnson Retina Center, University of Washington, Seattle, United States of America.
Background: Current clinical sequencing methods cannot effectively detect DNA methylation and allele-specific variation to provide parent-of-origin information from the proband alone. Parent-of-origin effects can lead to differential disease and the inability to assign this in de novo cases limits prognostication in the majority of affected individuals with retinoblastoma, a hereditary cancer with suspected parent-of-origin effects.
Methods: To directly assign parent-of-origin in retinoblastoma patients, genomic DNA was extracted from blood samples for sequencing using a programmable, targeted single-molecule long-read DNA genomic and epigenomic approach.
Cancer Med
December 2024
Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of California San Diego/Rady Children's Hospital San Diego, San Diego, California, USA.
Background: Measures to control COVID-19 transmission disrupted childhood cancer care. Data on the effects of the COVID-19 pandemic on childhood cancer mortality are lacking. This study describes the impact of the pandemic on childhood cancer early-mortality (≤ 24 months).
View Article and Find Full Text PDFHarefuah
December 2024
Ophthalmology Department, Shaare Tzedek Medical Center.
Malignant tumors of the eye are uncommon, yet of great importance due to their effect on the patient's life and vision. The most common primary intraocular tumors are retinoblastoma in children and uveal melanoma in adults. The most common periocular tumor is basal cell carcinoma in eyelids.
View Article and Find Full Text PDFSemin Cancer Biol
December 2024
Department of Hepatobiliary and Pancreatic Surgery, Medical Research Institute, Frontier Science Center of Immunology and Metabolism, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China; Taikang Center for Life and Medical Sciences, Wuhan University, Wuhan 430071, China; Hubei Key Laboratory of Tumor Biological Behavior/Hubei Provincial Clinical Research Center for Cancer, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China. Electronic address:
The Retinoblastoma (RB) protein is crucial for regulating gene transcription and chromatin remodeling, impacting cell cycle progression, cellular senescence, and tumorigenesis. Cellular senescence, characterized by irreversible growth arrest and phenotypic alterations, serves as a vital barrier against tumor progression and age-related diseases. RB is crucial in mediating senescence and tumor suppression by modulating the RB-E2F pathway and cross talking with other key senescence effectors such as p53 and p16.
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