Anxiety, depression and fear of cancer recurrence in uveal melanoma survivors and ophthalmologist/oncologist communication during survivorship in France - protocol of a prospective observational mixed-method study.

Background: Quality of life (QoL) in patients undergoing surveillance for uveal melanoma (UM) can be affected by psychological sequelae. Fear of cancer recurrence (FCR) may be acute especially when prognostication indicates an increased risk of metastatic recurrence. Communication with an ophthalmologist or oncologist can then play a key role in impacting QoL.

A Clinico-Genetic Score Incorporating Disease-Free Intervals and Chromosome 8q Copy Numbers: A Novel Prognostic Marker for Recurrence and Survival Following Liver Resection in Patients with Liver Metastases of Uveal Melanoma.

Surgical treatment of liver metastases of uveal melanoma (LMUM) could be proposed for selected patients. This retrospective study examined the prognostic significance of the genetic profiles of liver metastases after LMUM resection. A total of 86 patients treated with resection for LMUM, who underwent genetic analysis of liver metastasis, were included.

Higher subfoveal choroidal thickness in choroidal melanomas than in choroidal nevi.

Purpose: To compare subfoveal choroidal thickness (SFCT) between eyes with choroidal melanoma and choroidal nevi.

Methods: Retrospective study of 126 consecutive patients in a tertiary ocular oncology center. Eyes with tumors located less than two disc-diameters from the fovea were excluded.

Diffuse Infiltrating Retinoblastoma with Anterior Chamber Involvement: Conservative Management and Identification of Alterations in Aqueous Humor.

Introduction: The aim of the study was to describe the successful conservative management of diffuse infiltrating retinoblastoma (DIR). Identification of pathogenic variant was done after cell-free DNA (cfDNA) analysis in aqueous humor.

Case Presentation: Herein, we report 2 patients with unilateral, non-familial DIR with anterior and posterior involvement.

Optical Density Ratio of Subretinal Fluid in Choroidal Melanomas Versus Choroidal Naevi Assessed by Optical Coherence Tomography.

Purpose: The purpose of this study was to determine whether optical density ratio (ODR) of subretinal fluid (SRF) on optical coherence tomography (OCT) differs between choroidal naevi and melanomas.

Methods: One hundred ninety-nine patients (one eye per patient) presenting choroidal melanoma or choroidal naevus with SRF on OCT, evaluated between February and June 2019, were retrospectively included. Other retinal conditions, opaque media, and low-quality OCT were excluded.

Germline retrogene insertion in gene involved in cancer predisposition.

About half of the human genome is composed of repeated sequences derived from mobile elements, mainly retrotransposons, generally without pathogenic effect. Familial forms of retinoblastoma are caused by germline pathogenic variants in gene. Here, we describe a family with retinoblastoma affecting a father and his son.

Tumour growth rate improves tumour assessment and first-line systemic treatment decision-making for immunotherapy in patients with liver metastatic uveal melanoma.

Background: The RECIST-based response variably matches the clinical benefit of systemic therapies for liver metastatic uveal melanoma (LMUM). The aims were to determine whether the tumour growth rate (TGR) can help predict the survival in patients with LMUM and to provide information for the management of first-line systemic treatment.

Methods: This retrospective study included 147 (training: n = 110, validation: n = 37) patients with LMUM treated with first-line systemic treatment between 2010 and 2021.

Highly Sensitive Detection Method of Retinoblastoma Genetic Predisposition and Biomarkers.

Retinoblastoma is a malignant tumor of the infant retina. Nearly half of patients are predisposed to retinoblastoma by a germline RB1 pathogenic variant. Nonhereditary retinoblastoma is mainly caused by inactivation of both RB1 alleles at a somatic level.

Current Indications of Secondary Enucleation in Retinoblastoma Management: A Position Paper on Behalf of the European Retinoblastoma Group (EURbG).

Secondary enucleation (SE) puts an irreversible end to eye-preserving therapies, whenever their prolongation is expected to violate the presumed state of metastatic grace. At present, it must be acknowledged that clear criteria for SE are missing, leading to empiric and subjective indications commonly related to disease progression or relapse, disease persistence masking the optic nerve head or treatment-related complications obscuring the fundus view. This absence of evidence-based consensus regarding SE is explained by the continuously moving frontiers of the conservative management as a result of diagnostic and therapeutic advances, as well as by the lack of studies sufficiently powered to accurately stratify the risk of metastasis in conservatively treated patients.

Selective Ophthalmic Artery Chemotherapy with Melphalan in the Management of Unilateral Retinoblastoma: A Prospective Study.

Purpose: To determine prospectively the efficacy and to assess potential side effects of melphalan selective ophthalmic artery chemotherapy (SOAC) as first-line treatment for unilateral retinoblastoma.

Design: Phase 2 nonrandomized, prospective study.

Participants: Patients with unilateral retinoblastoma group B, C, or D of the International Classification for Intraocular Retinoblastoma (IRC).

Prospective phase II study of children affected by bilateral intraocular retinoblastoma with macular involvement of both eyes or in the only preserved eye. Macular tumor control, eye preservation rate, and visual outcome.

Background: Conservative treatments of intraocular retinoblastoma often consist of chemotherapy and focal treatments. The protocols vary and currently may combine two or three drugs, with different number of cycles, associated to the ocular treatments. In case of macular/paramacular involvement, tumor location and retinal scars induced by focal treatments often have a major negative impact on final visual outcome.

Association Between Genotype and Phenotype in Consecutive Unrelated Individuals With Retinoblastoma.

Importance: Retinoblastoma (RB) is the most common pediatric intraocular neoplasm. RB is a complex model in which atypical pathogenic variants, modifier genes, imprinting, and mosaicism are known to be associated with the phenotype. In-depth understanding of RB therefore requires large genotype-phenotype studies.

Diffuse Iris Melanoma: Conservative Treatment with Proton Beam Therapy after Limbal Stem Cell Preservation or Enucleation?

Objectives: Iris melanomas represent 2-3% of uveal melanomas; the diffuse variant accounts for approximately 10% of all iris melanomas. Different treatment modalities for diffuse iris melanomas (DIM) have been proposed depending on the local status as well as the age and general condition of the patient.

Methods: This study is a single-centre retrospective case series describing the diagnosis, treatments and outcomes of DIM.

Management of advanced uni- or bilateral retinoblastoma with macroscopic optic nerve invasion.

Background: Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high-risk patients.

Methods: Retrospective mono-institutional clinical, radiological, and histological review of patients with uni- or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed.

Osteosarcoma without prior retinoblastoma related to RB1 low-penetrance germline pathogenic variants: A novel type of RB1-related hereditary predisposition syndrome?

Background: Retinoblastoma (Rb) is a rare intraocular malignant tumor in children with high overall survival. Predisposition to Rb is linked to RB1 germline mutations with high penetrance, but rare RB1 low-penetrance variants are also known. Rb survivors are at risk of second primary malignancies (SPMs), mostly osteosarcoma and soft-tissue sarcoma.

Comparative Cytogenetic Abnormalities in Paired Choroidal Melanoma Samples Obtained Before and After Proton Beam Irradiation by Transscleral Fine-Needle Aspiration Biopsy and Endoresection.

This study compared the cytogenetic profiles of choroidal melanoma samples retrieved before and after proton beam irradiation. Twenty-four consecutive patients who underwent both fine-needle aspiration biopsy (FNAB) during tantalum clip positioning, and endoresection within three months of irradiation, were retrospectively included. Chromosome alterations were explored by array comparative genomic hybridization.

Long-Term Visual Outcomes for Small Uveal Melanoma Staged T1 Treated by Proton Beam Radiotherapy.

There is increasing evidence of the survival benefit of treating uveal melanoma in an early stage, however it is important to discuss with the patient the associated risk of visual loss. We investigated visual outcomes for uveal melanomas staged T1 (T1UM) treated by proton beam radiotherapy (PBR) as a function of their distance to fovea-optic disc. This retrospective study included a cohort of 424 patients with T1UM treated with PBR between 1991 and 2010 with at least a 5-year follow-up.

Development of a Prognostic Nomogram for Liver Metastasis of Uveal Melanoma Patients Selected by Liver MRI.

Patients with liver metastases of uveal melanoma (LMUM) die from their metastatic evolution within 2 years. We established a nomogram to choose a treatment adapted to life expectancy. From 2002 to 2013, we reviewed 224 patients with LMUM selected by liver MRI.

Iterative Variance Stabilizing Transformation Denoising of Spectral Domain Optical Coherence Tomography Images Applied to Retinoblastoma.

Background: Due to the presence of speckle Poisson noise, the interpretation of spectral domain-optical coherence tomography (SD-OCT) images frequently requires the use of data averaging to improve the signal-to-noise ratio. This implies long acquisition times and requires patient sedation in some cases. Iterative variance stabilizing transformation (VST) is a possible approach by which to remove speckle Poisson noise on single images.

TREATMENT OF EXUDATIVE CIRCUMSCRIBED CHOROIDAL HEMANGIOMA: Efficacy of Fractionated Proton Therapy (20 Gray Relative Biological Effectiveness in 8 Fractions).

Purpose: To evaluate treatment of circumscribed choroidal hemangioma by hyperfractionated proton beam therapy protocol (20 gray relative biological effectiveness in 8 fractions) on tumor control, attachment of retina and visual function.

Methods: Retrospective review of patients treated between January 2010 and April 2015 with at least 6 months of follow-up.

Results: Forty-three patients with exudative and symptomatic circumscribed choroidal hemangioma were included.

Prospective analysis of serious cardiorespiratory events in children during ophthalmic artery chemotherapy for retinoblastoma under a deep standardized anesthesia.

Background And Objective: Serious adverse cardiorespiratory events complicate super selective ophthalmic artery chemotherapy for retinoblastoma in anesthetized children. Their mechanism remains unclear but may be attributed to an autonomic nervous reflex induced by the catheter close to the ophthalmic artery. Inadequate depth of anesthesia during catheter stimulation might be an aggravating factor.

Intravitreal bevacizumab for neovascular glaucoma in uveal melanoma treated by proton beam therapy.

Background: To evaluate the efficacy of bevacizumab on reduction of the enucleation rate and control of intraocular pressure (IOP) in neovascular glaucoma (NVG)-complicating proton beam therapy for UM and to identify the determinants of the efficacy of bevacizumab.

Methods: Retrospective comparative study of patients with rubeosis following proton therapy for uveal melanoma. Patients were divided into two groups: a bevacizumab group and a control group which comprised two subgroups: panretinal photocoagulation (PRP)/cryotherapy and observation subgroups.

[Retinoblastoma].

Retinoblastoma. Retinoblastoma is the most frequent malignant intraocular tumour of childhood. It is a rare disease occurring in very young children.

Eye Salvage with Combination of Intravitreal and Intracameral Melphalan Injection for Recurrent Retinoblastoma with Anterior Chamber Involvement: Report of a Case.

Aims: Herein we report successful eye salvage following combined intravitreal and intracameral administration of melphalan for anterior chamber recurrence of retinoblastoma.

Methods: A 12-month-old boy was referred with a sporadic bilateral retinoblastoma staged as group D and V in the right eye and group E and V in the left eye. Treatment initially involved 6 cycles of 3 drugs followed by focal treatments.