Metastatic conjunctival melanoma: a multicentre international study.

Background: To reveal clinical findings related to metastatic conjunctival melanoma.

Methods: 10 ophthalmic oncology centres (9 countries and 4 continents) shared data to create a large clinical case series. The main outcome measures were the incidence and cumulative risk of systemic metastasis, study mortality rates and Kaplan-Meier patient mortality after developing conjunctival melanoma metastasis.

Digital retinoblastoma documentation supports care and research.

Introduction: In 1948, the indirect ophthalmoscope revealed full intra-ocular retinoblastoma, documented on paper with coloured pencils. At SickKids, eCancerCare (eCC) digital drawings and timeline have facilitated patient care, education, and research.

Methods: Each child's eCC timeline shows treatments and dates.

Retinoblastoma caused by an RB1 variant with unusually low penetrance in a Danish family.

Retinoblastoma is the most common eye cancer in children. It is caused by pathogenic alterations of both alleles of the tumor suppressor gene RB1. In heritable retinoblastoma, a constitutional RB1 variant predisposes the cells to tumor formation, and loss of the other allele is a prerequisite for the development of retinoblastoma.

Orbital Complications and Cost Analysis of Enucleation by Myoconjunctival versus Conventional Techniques for Retinoblastoma.

Introduction: The aim of the study was to compare complication rates and hospital costs of myoconjunctival versus conventional enucleation techniques in retinoblastoma.

Methods: This retrospective cohort and cost analysis reviewed patients with retinoblastoma treated by primary or secondary enucleation between 2003 and 2021 and a minimum 6-month follow-up. Cases were reviewed for three postsurgical complications: chronic conjunctivitis, implant exposure/extrusion, and cellulitis.

Adjuvant Chemotherapy Improves Survival for Children With Massive Choroidal Invasion of Retinoblastoma.

Purpose: The purpose of this study was to investigate the effect of adjuvant chemotherapy on outcomes of children with massive choroidal invasion (MCI).

Methods: In this study, we reviewed the 5-year relapse-free survival (RFS) and overall survival (OS) of children diagnosed with MCI, managed with or without adjuvant chemotherapy. Excluded were children with additional other high-risk features (post-laminar optic nerve invasion, scleral invasion, or overt extraocular disease).

Nitric oxide suppression by secreted frizzled-related protein 2 drives retinoblastoma.

Retinoblastoma is a cancer of the infant retina primarily driven by loss of the Rb tumor suppressor gene, which is undruggable. Here, we report an autocrine signaling, mediated by secreted frizzled-related protein 2 (SFRP2), which suppresses nitric oxide and enables retinoblastoma growth. We show that coxsackievirus and adenovirus receptor (CXADR) is the cell-surface receptor for SFRP2 in retinoblastoma cells; that CXADR functions as a "dependence receptor," transmitting a growth-inhibitory signal in the absence of SFRP2; and that the balance between SFRP2 and CXADR determines nitric oxide production.

A typical anterior retinoblastoma: diagnosis by aqueous humor cell-free DNA analysis.

Background: Aqueous humor from eyes with active retinoblastoma contains tumor-derived cell-free DNA.

Materials And Methods: Single retrospective case report.

Results: A 13-year-old girl with acute right eye pain and redness was diagnosed with hypertensive anterior uveitis.

The Retinoblastoma Research Booklet: A Catalyst for Patient Involvement in Retinoblastoma Research.

Peer-to-peer recruitment efforts are important in generating interest and participation of patients as partners in research but difficult to sustain when face-to-face interactions are limited. The Retinoblastoma Research and You! booklet, co-developed by patients, researchers and health professionals, serves as a guide for patient engagement in research while retaining an element of personalization. The Retinoblastoma Research and You! booklet was developed through two virtual workshops to iterate and finalize the booklet design and content.

EMPOWER Retinoblastoma: Engaging Patient Partners in Solving the Top 10 Priorities for Eye Cancer Research in Canada.

While it is recognized that research priorities should reflect and integrate the perspectives and needs of patients along with those of health professionals and researchers, it remains challenging to actualize such priorities into tangible research projects. Targeted dissemination is required to catalyze research on these priorities. To create awareness of and inspire action toward actualizing the top 10 retinoblastoma research priorities in Canada, Canadian Retinoblastoma Research Advisory Board (CRRAB) members developed a wide range of dissemination tools and processes.

High-risk Pathologic Features Based on Presenting Findings in Advanced Intraocular Retinoblastoma: A Multicenter, International Data-Sharing American Joint Committee on Cancer Study.

Purpose: To determine the value of clinical features for advanced intraocular retinoblastoma as defined by the eighth edition of the American Joint Committee on Cancer (AJCC) cT3 category and AJCC Ophthalmic Oncology Task Force (OOTF) Size Groups to predict the high-risk pathologic features.

Design: International, multicenter, registry-based retrospective case series.

Participants: Eighteen ophthalmic oncology centers from 13 countries over 6 continents shared evaluations of 942 eyes enucleated as primary treatment for AJCC cT3 and, for comparison, cT2 retinoblastoma.

Metachronous, non-pineal, trilateral retinoblastoma in a patient with a seemingly reduced-expressivity germline deletion.

The clinical course of trilateral retinoblastoma can be unpredictable, and expressivity of germline variants may vary during development. We describe an unexpected fatal case of trilateral retinoblastoma with an intracranial tumor in an unusual location and discuss genetic copy number analyses as a useful diagnostic tool with therapeutic potential.

Pediatric Cataract Surgery Following Treatment for Retinoblastoma: A Case Series and Systematic Review.

Purpose: To determine the visual and refractive outcomes and the ocular and systemic complications of cataract surgery in eyes treated for retinoblastoma.

Design: Retrospective consecutive case series and systematic review.

Methods: Children <18 years of age with retinoblastoma who underwent surgery for secondary cataract between 2000 and 2020 were reviewed.

Retinoblastoma Survival Following Primary Enucleation by AJCC Staging.

Primary enucleation of the eye with retinoblastoma is a widely accessible, life-saving treatment for retinoblastoma. This study evaluated the survival of patients following primary enucleation based on AJCC 8th edition staging. Included were 700 consecutive patients (700 eyes) treated with primary enucleation at 29 Chinese treatment centers between 2006 and 2015.

Tylectomy Safety in Salvage of Eyes with Retinoblastoma.

Intraocular surgery is tabooed in retinoblastoma management, due to the concern of lethal extraocular spread. We reviewed the outcomes of consecutive children with intraocular retinoblastoma diagnosed at 29 Chinese centers between 2012-2014. We compared the outcomes of three categories of treatment: eye salvage including tylectomy (Group I), eye salvage without tylectomy (Group II), and primary enucleation (Group III).

Natural History of Untreated Retinoblastoma.

Treatment abandonment is a leading cause of death in children with retinoblastoma worldwide. We studied children who abandoned treatment upfront at diagnosis to delineate the natural history of untreated retinoblastoma. Studied were children who received no treatment, diagnosed between 2007 and 2017 at 29 Chinese centers.

Retinoblastoma seeds: impact on American Joint Committee on Cancer clinical staging.

Aim: To investigate whether the American Joint Committee on Cancer (AJCC) clinical category cT2b needs to be subclassified by the type and distribution of retinoblastoma (RB) seeding.

Methods: Multicentre, international registry-based data were collected from RB centres enrolled between January 2001 and December 2013. 1054 RB eyes with vitreous or subretinal seeds from 18 ophthalmic oncology centres, in 13 countries within six continents were analysed.

germline mutation spectrum and clinical features in patients with unilateral retinoblastomas.

: Retinoblastoma is the most common intraocular cancer in children in which above 90% of bilateral cases and 10-25% of unilateral cases have germline mutations. We summarized the spectrum of germline mutations and the clinical manifestations of unilateral retinoblastomas to guide clinical treatments.: Two hundred and sixty-three unrelated patients with unilateral retinoblastoma and their parents were included between February 2014 and August 2020.

Clinical audit of retinoblastoma management: a retrospective single-institution study.

Objective: The primary aim of this study was to identify the frequency of death, metastasis, enucleation, and use of external beam radiation therapy (EBRT) among retinoblastoma patients. The secondary aim was to determine whether any events were associated with suboptimal clinical management to identify areas for clinical care improvement.

Methods: Patients diagnosed with retinoblastoma between January 1, 2000, and December 31, 2015, at The Hospital for Sick Children were included.