Purpose: Trilateral retinoblastoma (TRB), intracranial neoplasm in heritable retinoblastoma patients, is a very rare fatal disease. Many ocular oncology centers conduct routine screening of retinoblastoma patients by brain imaging. Nevertheless, there is a debate regarding its ability to prolong TRB patients' survival and the number-needed-to-treat.
View Article and Find Full Text PDFBackground: To evaluate the clinical presentation, pathological features and outcomes of retinoblastoma based on the race of origin in a global cohort of patients.
Methods: Retrospective collaborative study of 1426 patients who underwent primary enucleation for retinoblastoma.
Results: Patients were grouped into Caucasians (n = 231, 16%), Asians (n = 841, 59%), Hispanics (n = 226, 16%), Arabs (n = 96, 7%) and Others (Africans, African Americans, Indigenous Australians; n = 32, 2%) cohorts.
Background: Observational studies should not be used to infer causation as they are prone to confounding factors, selection bias, and reverse causality. Many observational studies of labor analgesia treated epidurals as an independent exposure and concluded that "epidurals" cause dystocia, despite multiple randomized controlled trials showing no effect. We highlight this problem using reductio ad absurdum.
View Article and Find Full Text PDFPurpose: To compare the clinical outcomes of children with unilateral retinoblastoma (Rb) and high-risk histopathology features (HRHF) following upfront enucleation with/without adjuvant chemotherapy, and investigate cases locally considered non-HRHF but converted to a standardized HRHF definition.
Design: Retrospective multinational clinical cohort study.
Methods: Children with Rb who presented to 21 centers from 12 countries between 2011-2020, and underwent primary enucleation were recruited.
Anticancer Res
March 2022
Background/aim: Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. High serum levels of soluble IL-2 receptor (sIL-2R) have been reported in acute inflammations and metastatic cancers. This study evaluated the potential of high/increasing sIL-2R levels in predicting metastases.
View Article and Find Full Text PDFObjectives: To investigate the risk of developing central nervous system (CNS) lymphoma in patients with vitreoretinal lymphoma (VRL) presenting with unilateral versus (vs.) bilateral ocular involvement.
Methods: Retrospective, multicentre cohort study from January 1, 1984 to December 31, 2020.
Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL).
Methods: Literature was reviewed for reports supporting the diagnosis of VRL. A questionnaire (Delphi 1 round) was distributed to 28 participants.
Vitreo-retinal lymphoma (VRL) is the most common intraocular lymphoma and is highly associated with central nervous system (CNS) lymphoma (CNSL), both posing a therapeutic challenge. We investigated patients' characteristics, efficacy and safety of intravitreal methotrexate (MTX) injections and their outcomes over 20 years. The records of 129 patients diagnosed between 1997 and 2018 were retrospectively reviewed.
View Article and Find Full Text PDFBackground: Driving is a visually intensive task. In Cameroon, where the burden of road traffic deaths is high, visual assessment is not universally performed before the issuance of driver licenses. This study aims to assess the visual status of commercial drivers (CDs) in the southwestern region of Cameroon, and to find its relation to road traffic crashes (RTCs).
View Article and Find Full Text PDFObjective: Von Hippel-Lindau (VHL) syndrome is a rare and complex disease. In 1996, we described a 3 generation VHL 2A kindred with 11 mutation carriers. We aim to share our experience regarding the long-term follow-up of this family and the management of all our other VHL patients focusing on frequently encountered neuroendocrine neoplasms: pheochromocytoma/paraganglioma and pancreatic neuroendocrine neoplasms (PNEN).
View Article and Find Full Text PDFImportance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.
View Article and Find Full Text PDFPurpose: To evaluate the efficacy of the different diagnostic tests for vitreoretinal lymphoma (VRL).
Methods: A cohort of 150 patients with a presumed diagnosis of VRL. Vitrectomy samples were analysed for cytology, monoclonality [polymerase chain reaction (PCR)] and cytokine levels, and anterior chamber taps were analysed for cytokine levels.
Background: Monocular vision has been found to have a negative effect on children's motion processing and motor functions. Yet, knowledge of motor function of survivors of retinoblastoma (RB) with monocular vision (due to enucleation, for example) is limited. This study examined motor function and its relationship to visual-related and health-related quality of life (HRQOL) in survivors of RB with monocular vision.
View Article and Find Full Text PDFThe 2018 Ocular Oncogenesis and Oncology Conference was held through a partnership of the Association for Research in Vision and Ophthalmology (ARVO) and the Champalimaud Foundation. Twenty-one experts from international ocular oncology centers, from the Champalimaud Clinical Centre and the Champalimaud Foundation Cancer Research Program, and from patient advocacy organizations, delivered lectures on subjects that ranged from global ocular oncology, to basic research in mechanisms of ocular malignancy, to clinical research in ocular cancers, and to anticipated future developments in the area. The scientific program of the conference covered a broad range of ocular tumors-including uveal melanoma, retinoblastoma, ocular surface tumors, and adnexal and intraocular lymphomas-and pathogenesis and management were deliberated in the context of the broader systemic cancer discipline.
View Article and Find Full Text PDFUveal melanoma (UM) is the most frequent intraocular tumor in adult patients. When metastases occur, systemic therapy with alkylating agents (fotemustine or dacarbazine (DTIC)) has shown only modest efficacy. The common chemotherapeutic drug doxorubicin (DOX) is not used to treat metastatic UM (mUM).
View Article and Find Full Text PDFBackground: Mucin 13 (MUC13) is a cell surface glycoprotein aberrantly expressed in a variety of epithelial carcinomas. Thus far, the role of MUC13 in various diseases remains elusive. To the best of our knowledge, this is the first study to examine the potential of MUC13 as a serum biomarker in a variety of carcinomas and other conditions.
View Article and Find Full Text PDFIntroduction: Retinoblastoma (RB) is a malignant tumor presenting in the eyes of infants and children, which endangers life, the eye and vision. The treatment of RB has undergone marked changes in recent years, and great progress has been made in our ability to preserve eyes. Over the last three decades most Israeli patients with RB have been treated in the National Specialty Ocular Oncology Service at the Hadassah-Hebrew University Medical Center in Jerusalem.
View Article and Find Full Text PDFInvest Ophthalmol Vis Sci
February 2018
Purpose: Retinoblastomas' growth rate is dependent on their ability to induce neovascularization. Leucine-rich α-2-glycoprotein-1 (LRG-1) was recently reported to be upregulated in human retinal disease with neovascular pathology. The purpose of the study was to determine LRG-1 expression in human retinoblastoma and to correlate it with clinical and histopathologic parameters and to assess how its expression correlates with vascular endothelial growth factor (VEGF) expression.
View Article and Find Full Text PDFPurpose: Patients with uveal melanoma (UM) undergo lifelong follow-up as metastases can occur more than 20 years after diagnosis. Little is known about the quality of life (QoL) of UM survivors over such an extended period. To investigate their QoL, we used various estimating factors.
View Article and Find Full Text PDFThe purpose of this study was to describe the distribution of Muller cell within the peripapillary retinal nerve fiber layer (RNFL) in human eyes. Eleven unpaired normal postmortem eyes were recruited into this study. Each eye was sectioned using the "umbrella technique" to obtain a concentric peripapillary ring centered on the optic disc, with a diameter of 3.
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